AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPsychiatry and PsychologyPhenomena and ProcessesInformation ScienceHealth Care
Myeloproliferative DisordersPolycythemia VeraPrimary MyelofibrosisJanus Kinase 2Thrombocythemia, EssentialThrombocytosisReceptors, ThrombopoietinLeukemia, Myeloid, Chronic, Atypical, BCR-ABL NegativeLeukemia, Myelomonocytic, ChronicSplenomegalyHematopoiesis, ExtramedullaryLeukemia, Neutrophilic, ChronicLeukemia, Myelogenous, Chronic, BCR-ABL PositiveLeukemia, Myelomonocytic, JuvenileBone MarrowMyelodysplastic SyndromesHematopoietic Stem CellsLeukemia, Megakaryoblastic, AcuteGranulocytesDown SyndromeFusion Proteins, bcr-ablMegakaryocytesReceptor, Fibroblast Growth Factor, Type 1Leukemia, MyeloidMutationPolycythemiaMyelodysplastic-Myeloproliferative DiseasesHepatomegalyTranslocation, GeneticHypereosinophilic SyndromeLeukocytosisChromosomes, Human, Pair 8Budd-Chiari SyndromeGenes, ablThrombopoietinLeukemia, Myeloid, AcuteHematopoiesisMutation, MissenseOncogene Proteins, FusionHydroxyureaChromosomes, Human, Pair 20Blast CrisisX Chromosome InactivationBipolar DisorderMyelopoiesisColony-Forming Units AssayChromosomes, Human, Pair 5BenzamidesAnemia, Refractory, with Excess of BlastsBone Marrow CellsPlatelet CountChronic DiseaseBone Marrow NeoplasmsGATA1 Transcription FactorMental DisordersPhiladelphia ChromosomeHydrops FetalisLeukemiaBlood Cell CountPyrimidinesPiperazinesHematologic DiseasesJanus KinasesChromosomes, Human, XAnxiety DisordersDNA Mutational AnalysisMyeloid Progenitor CellsMood DisordersMyeloid CellsPoint MutationChromosomes, Human, Pair 13KaryotypingProtein-Tyrosine KinasesGene Expression Regulation, LeukemicValineProto-Oncogene ProteinsErythroid Precursor CellsAmino Acid SubstitutionHematologic NeoplasmsCell Transformation, NeoplasticReceptor, Platelet-Derived Growth Factor betaBone Marrow TransplantationReceptors, Fibroblast Growth FactorSplenectomyNeurofibromin 1Chromosomes, Human, Pair 9Clone CellsCore Binding Factor Alpha 2 SubunitSignal TransductionDiagnostic and Statistical Manual of Mental DisordersIn Situ Hybridization, FluorescencePhenotypePolymerase Chain ReactionDisease Models, AnimalBlood PlateletsSarcoma Viruses, MurineChromosome AberrationsReceptors, CytokineErythropoietinAntigens, CD34
Myeloproliferative Disorders
Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE.
Polycythemia Vera
A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs.
Primary Myelofibrosis
A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone.
Janus Kinase 2
A Janus kinase subtype that is involved in signaling from GROWTH HORMONE RECEPTORS; PROLACTIN RECEPTORS; and a variety of CYTOKINE RECEPTORS such as ERYTHROPOIETIN RECEPTORS and INTERLEUKIN RECEPTORS. Dysregulation of Janus kinase 2 due to GENETIC TRANSLOCATIONS have been associated with a variety of MYELOPROLIFERATIVE DISORDERS.
Thrombocythemia, Essential
A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.
Thrombocytosis
Increased numbers of platelets in the peripheral blood. (Dorland, 27th ed)
Receptors, Thrombopoietin
Cell surface receptors that are specific for THROMBOPOIETIN. They signal through interaction with JANUS KINASES such as JANUS KINASE 2.
Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative
A myelodysplastic/myeloproliferative disorder characterized by myelodysplasia associated with bone marrow and peripheral blood patterns similar to CHRONIC MYELOID LEUKEMIA, but cytogenetically lacking a PHILADELPHIA CHROMOSOME or bcr/abl fusion gene (GENES, ABL).
Leukemia, Myelomonocytic, Chronic
A myelodysplastic-myeloproliferative disease characterized by monocytosis, increased monocytes in the bone marrow, variable degrees of dysplasia, but an absence of immature granulocytes in the blood.
Splenomegaly
Enlargement of the spleen.
Hematopoiesis, Extramedullary
The formation and development of blood cells outside the BONE MARROW, as in the SPLEEN; LIVER; or LYMPH NODES.
Leukemia, Neutrophilic, Chronic
A rare myeloproliferative disorder that is characterized by a sustained, mature neutrophilic leukocytosis. No monocytosis, EOSINOPHILIA, or basophilia is present, nor is there a PHILADELPHIA CHROMOSOME or bcr-abl fusion gene (GENES, ABL).
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Clonal hematopoetic disorder caused by an acquired genetic defect in PLURIPOTENT STEM CELLS. It starts in MYELOID CELLS of the bone marrow, invades the blood and then other organs. The condition progresses from a stable, more indolent, chronic phase (LEUKEMIA, MYELOID, CHRONIC PHASE) lasting up to 7 years, to an advanced phase composed of an accelerated phase (LEUKEMIA, MYELOID, ACCELERATED PHASE) and BLAST CRISIS.
Leukemia, Myelomonocytic, Juvenile
A leukemia affecting young children characterized by SPLENOMEGALY, enlarged lymph nodes, rashes, and hemorrhages. Traditionally classed as a myeloproliferative disease, it is now considered a mixed myeloproliferative-mylelodysplastic disorder.
Bone Marrow
The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.
Myelodysplastic Syndromes
Clonal hematopoietic stem cell disorders characterized by dysplasia in one or more hematopoietic cell lineages. They predominantly affect patients over 60, are considered preleukemic conditions, and have high probability of transformation into ACUTE MYELOID LEUKEMIA.
Hematopoietic Stem Cells
Progenitor cells from which all blood cells derive.
Leukemia, Megakaryoblastic, Acute
An acute myeloid leukemia in which 20-30% of the bone marrow or peripheral blood cells are of megakaryocyte lineage. MYELOFIBROSIS or increased bone marrow RETICULIN is common.
Granulocytes
Leukocytes with abundant granules in the cytoplasm. They are divided into three groups according to the staining properties of the granules: neutrophilic, eosinophilic, and basophilic. Mature granulocytes are the NEUTROPHILS; EOSINOPHILS; and BASOPHILS.
Down Syndrome
A chromosome disorder associated either with an extra chromosome 21 or an effective trisomy for chromosome 21. Clinical manifestations include hypotonia, short stature, brachycephaly, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, Simian crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213)
Fusion Proteins, bcr-abl
Translation products of a fusion gene derived from CHROMOSOMAL TRANSLOCATION of C-ABL GENES to the genetic locus of the breakpoint cluster region gene on chromosome 22. Several different variants of the bcr-abl fusion proteins occur depending upon the precise location of the chromosomal breakpoint. These variants can be associated with distinct subtypes of leukemias such as PRECURSOR CELL LYMPHOBLASTIC LEUKEMIA-LYMPHOMA; LEUKEMIA, MYELOGENOUS, CHRONIC, BCR-ABL POSITIVE; and NEUTROPHILIC LEUKEMIA, CHRONIC.
Megakaryocytes
Very large BONE MARROW CELLS which release mature BLOOD PLATELETS.
Receptor, Fibroblast Growth Factor, Type 1
A fibroblast growth factor receptor with specificity for FIBROBLAST GROWTH FACTORS; HEPARAN SULFATE PROTEOGLYCAN; and NEURONAL CELL ADHESION MOLECULES. Several variants of the receptor exist due to multiple ALTERNATIVE SPLICING of its mRNA. Fibroblast growth factor receptor 1 is a tyrosine kinase that transmits signals through the MAP KINASE SIGNALING SYSTEM.
Leukemia, Myeloid
Form of leukemia characterized by an uncontrolled proliferation of the myeloid lineage and their precursors (MYELOID PROGENITOR CELLS) in the bone marrow and other sites.
Mutation
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
Polycythemia
An increase in the total red cell mass of the blood. (Dorland, 27th ed)
Myelodysplastic-Myeloproliferative Diseases
Clonal myeloid disorders that possess both dysplastic and proliferative features but are not properly classified as either MYELODYSPLASTIC SYNDROMES or MYELOPROLIFERATIVE DISORDERS.
Hepatomegaly
Enlargement of the liver.
Translocation, Genetic
A type of chromosome aberration characterized by CHROMOSOME BREAKAGE and transfer of the broken-off portion to another location, often to a different chromosome.
Hypereosinophilic Syndrome
A heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and associated organ system dysfunction, including the heart, central nervous system, kidneys, lungs, gastrointestinal tract, and skin. There is a massive increase in the number of EOSINOPHILS in the blood, mimicking leukemia, and extensive eosinophilic infiltration of the various organs.
Leukocytosis
A transient increase in the number of leukocytes in a body fluid.
Chromosomes, Human, Pair 8
A specific pair of GROUP C CHROMOSOMES of the human chromosome classification.
Budd-Chiari Syndrome
A condition in which the hepatic venous outflow is obstructed anywhere from the small HEPATIC VEINS to the junction of the INFERIOR VENA CAVA and the RIGHT ATRIUM. Usually the blockage is extrahepatic and caused by blood clots (THROMBUS) or fibrous webs. Parenchymal FIBROSIS is uncommon.
Genes, abl
Retrovirus-associated DNA sequences (abl) originally isolated from the Abelson murine leukemia virus (Ab-MuLV). The proto-oncogene abl (c-abl) codes for a protein that is a member of the tyrosine kinase family. The human c-abl gene is located at 9q34.1 on the long arm of chromosome 9. It is activated by translocation to bcr on chromosome 22 in chronic myelogenous leukemia.
Thrombopoietin
A humoral factor that stimulates the production of thrombocytes (BLOOD PLATELETS). Thrombopoietin stimulates the proliferation of bone marrow MEGAKARYOCYTES and their release of blood platelets. The process is called THROMBOPOIESIS.
Leukemia, Myeloid, Acute
Clonal expansion of myeloid blasts in bone marrow, blood, and other tissue. Myeloid leukemias develop from changes in cells that normally produce NEUTROPHILS; BASOPHILS; EOSINOPHILS; and MONOCYTES.
Hematopoiesis
The development and formation of various types of BLOOD CELLS. Hematopoiesis can take place in the BONE MARROW (medullary) or outside the bone marrow (HEMATOPOIESIS, EXTRAMEDULLARY).
Mutation, Missense
A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)
Oncogene Proteins, Fusion
The GENETIC TRANSLATION products of the fusion between an ONCOGENE and another gene. The latter may be of viral or cellular origin.
Hydroxyurea
An antineoplastic agent that inhibits DNA synthesis through the inhibition of ribonucleoside diphosphate reductase.
Chromosomes, Human, Pair 20
A specific pair of GROUP F CHROMOSOMES of the human chromosome classification.
Blast Crisis
An advanced phase of chronic myelogenous leukemia, characterized by a rapid increase in the proportion of immature white blood cells (blasts) in the blood and bone marrow to greater than 30%.
X Chromosome Inactivation
A dosage compensation process occurring at an early embryonic stage in mammalian development whereby, at random, one X CHROMOSOME of the pair is repressed in the somatic cells of females.
Bipolar Disorder
A major affective disorder marked by severe mood swings (manic or major depressive episodes) and a tendency to remission and recurrence.
Myelopoiesis
Formation of MYELOID CELLS from the pluripotent HEMATOPOIETIC STEM CELLS in the BONE MARROW via MYELOID STEM CELLS. Myelopoiesis generally refers to the production of leukocytes in blood, such as MONOCYTES and GRANULOCYTES. This process also produces precursor cells for MACROPHAGE and DENDRITIC CELLS found in the lymphoid tissue.
Colony-Forming Units Assay
A cytologic technique for measuring the functional capacity of stem cells by assaying their activity.
Chromosomes, Human, Pair 5
One of the two pairs of human chromosomes in the group B class (CHROMOSOMES, HUMAN, 4-5).
Benzamides
BENZOIC ACID amides.
Anemia, Refractory, with Excess of Blasts
Chronic refractory anemia with granulocytopenia, and/or thrombocytopenia. Myeloblasts and progranulocytes constitute 5 to 40 percent of the nucleated marrow cells.
Bone Marrow Cells
Cells contained in the bone marrow including fat cells (see ADIPOCYTES); STROMAL CELLS; MEGAKARYOCYTES; and the immediate precursors of most blood cells.
Platelet Count
The number of PLATELETS per unit volume in a sample of venous BLOOD.
Chronic Disease
Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care. (Dictionary of Health Services Management, 2d ed)
Bone Marrow Neoplasms
Neoplasms located in the bone marrow. They are differentiated from neoplasms composed of bone marrow cells, such as MULTIPLE MYELOMA. Most bone marrow neoplasms are metastatic.
GATA1 Transcription Factor
A GATA transcription factor that is specifically expressed in hematopoietic lineages and plays an important role in the CELL DIFFERENTIATION of ERYTHROID CELLS and MEGAKARYOCYTES.
Mental Disorders
Psychiatric illness or diseases manifested by breakdowns in the adaptational process expressed primarily as abnormalities of thought, feeling, and behavior producing either distress or impairment of function.
Philadelphia Chromosome
An aberrant form of human CHROMOSOME 22 characterized by translocation of the distal end of chromosome 9 from 9q34, to the long arm of chromosome 22 at 22q11. It is present in the bone marrow cells of 80 to 90 per cent of patients with chronic myelocytic leukemia (LEUKEMIA, MYELOGENOUS, CHRONIC, BCR-ABL POSITIVE).
Hydrops Fetalis
Abnormal accumulation of serous fluid in two or more fetal compartments, such as SKIN; PLEURA; PERICARDIUM; PLACENTA; PERITONEUM; AMNIOTIC FLUID. General fetal EDEMA may be of non-immunologic origin, or of immunologic origin as in the case of ERYTHROBLASTOSIS FETALIS.
Leukemia
A progressive, malignant disease of the blood-forming organs, characterized by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow. Leukemias were originally termed acute or chronic based on life expectancy but now are classified according to cellular maturity. Acute leukemias consist of predominately immature cells; chronic leukemias are composed of more mature cells. (From The Merck Manual, 2006)
Blood Cell Count
The number of LEUKOCYTES and ERYTHROCYTES per unit volume in a sample of venous BLOOD. A complete blood count (CBC) also includes measurement of the HEMOGLOBIN; HEMATOCRIT; and ERYTHROCYTE INDICES.
Pyrimidines
A family of 6-membered heterocyclic compounds occurring in nature in a wide variety of forms. They include several nucleic acid constituents (CYTOSINE; THYMINE; and URACIL) and form the basic structure of the barbiturates.
Piperazines
Hematologic Diseases
Disorders of the blood and blood forming tissues.
Janus Kinases
A family of intracellular tyrosine kinases that participate in the signaling cascade of cytokines by associating with specific CYTOKINE RECEPTORS. They act upon STAT TRANSCRIPTION FACTORS in signaling pathway referred to as the JAK/STAT pathway. The name Janus kinase refers to the fact the proteins have two phosphate-transferring domains.
Chromosomes, Human, X
The human female sex chromosome, being the differential sex chromosome carried by half the male gametes and all female gametes in humans.
Anxiety Disorders
Persistent and disabling ANXIETY.
DNA Mutational Analysis
Biochemical identification of mutational changes in a nucleotide sequence.
Myeloid Progenitor Cells
Stem cells derived from HEMATOPOIETIC STEM CELLS. Derived from these myeloid progenitor cells are the MEGAKARYOCYTES; ERYTHROID CELLS; MYELOID CELLS; and some DENDRITIC CELLS.
Mood Disorders
Those disorders that have a disturbance in mood as their predominant feature.
Myeloid Cells
The classes of BONE MARROW-derived blood cells in the monocytic series (MONOCYTES and their precursors) and granulocytic series (GRANULOCYTES and their precursors).
Point Mutation
A mutation caused by the substitution of one nucleotide for another. This results in the DNA molecule having a change in a single base pair.
Chromosomes, Human, Pair 13
A specific pair of GROUP D CHROMOSOMES of the human chromosome classification.
Karyotyping
Mapping of the KARYOTYPE of a cell.
Protein-Tyrosine Kinases
Protein kinases that catalyze the PHOSPHORYLATION of TYROSINE residues in proteins with ATP or other nucleotides as phosphate donors.
Gene Expression Regulation, Leukemic
Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control of gene action in leukemia.
Valine
A branched-chain essential amino acid that has stimulant activity. It promotes muscle growth and tissue repair. It is a precursor in the penicillin biosynthetic pathway.
Proto-Oncogene Proteins
Products of proto-oncogenes. Normally they do not have oncogenic or transforming properties, but are involved in the regulation or differentiation of cell growth. They often have protein kinase activity.
Erythroid Precursor Cells
The cells in the erythroid series derived from MYELOID PROGENITOR CELLS or from the bi-potential MEGAKARYOCYTE-ERYTHROID PROGENITOR CELLS which eventually give rise to mature RED BLOOD CELLS. The erythroid progenitor cells develop in two phases: erythroid burst-forming units (BFU-E) followed by erythroid colony-forming units (CFU-E); BFU-E differentiate into CFU-E on stimulation by ERYTHROPOIETIN, and then further differentiate into ERYTHROBLASTS when stimulated by other factors.
Amino Acid Substitution
The naturally occurring or experimentally induced replacement of one or more AMINO ACIDS in a protein with another. If a functionally equivalent amino acid is substituted, the protein may retain wild-type activity. Substitution may also diminish, enhance, or eliminate protein function. Experimentally induced substitution is often used to study enzyme activities and binding site properties.
Hematologic Neoplasms
Neoplasms located in the blood and blood-forming tissue (the bone marrow and lymphatic tissue). The commonest forms are the various types of LEUKEMIA, of LYMPHOMA, and of the progressive, life-threatening forms of the MYELODYSPLASTIC SYNDROMES.
Cell Transformation, Neoplastic
Cell changes manifested by escape from control mechanisms, increased growth potential, alterations in the cell surface, karyotypic abnormalities, morphological and biochemical deviations from the norm, and other attributes conferring the ability to invade, metastasize, and kill.
Receptor, Platelet-Derived Growth Factor beta
A PDGF receptor that binds specifically to the PDGF-B chain. It contains a protein-tyrosine kinase activity that is involved in SIGNAL TRANSDUCTION.
Bone Marrow Transplantation
The transference of BONE MARROW from one human or animal to another for a variety of purposes including HEMATOPOIETIC STEM CELL TRANSPLANTATION or MESENCHYMAL STEM CELL TRANSPLANTATION.
Receptors, Fibroblast Growth Factor
Specific molecular sites or structures on cell membranes that react with FIBROBLAST GROWTH FACTORS (both the basic and acidic forms), their analogs, or their antagonists to elicit or to inhibit the specific response of the cell to these factors. These receptors frequently possess tyrosine kinase activity.
Splenectomy
Surgical procedure involving either partial or entire removal of the spleen.
Neurofibromin 1
A protein found most abundantly in the nervous system. Defects or deficiencies in this protein are associated with NEUROFIBROMATOSIS 1, Watson syndrome, and LEOPARD syndrome. Mutations in the gene (GENE, NEUROFIBROMATOSIS 1) affect two known functions: regulation of ras-GTPase and tumor suppression.
Chromosomes, Human, Pair 9
A specific pair of GROUP C CHROMSOMES of the human chromosome classification.
Clone Cells
A group of genetically identical cells all descended from a single common ancestral cell by mitosis in eukaryotes or by binary fission in prokaryotes. Clone cells also include populations of recombinant DNA molecules all carrying the same inserted sequence. (From King & Stansfield, Dictionary of Genetics, 4th ed)
Core Binding Factor Alpha 2 Subunit
A transcription factor that dimerizes with the cofactor CORE BINDING FACTOR BETA SUBUNIT to form core binding factor. It contains a highly conserved DNA-binding domain known as the runt domain. Runx1 is frequently mutated in human LEUKEMIAS.
Signal Transduction
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
Diagnostic and Statistical Manual of Mental Disorders
Categorical classification of MENTAL DISORDERS based on criteria sets with defining features. It is produced by the American Psychiatric Association. (DSM-IV, page xxii)
In Situ Hybridization, Fluorescence
A type of IN SITU HYBRIDIZATION in which target sequences are stained with fluorescent dye so their location and size can be determined using fluorescence microscopy. This staining is sufficiently distinct that the hybridization signal can be seen both in metaphase spreads and in interphase nuclei.
Phenotype
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
Polymerase Chain Reaction
In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.
Disease Models, Animal
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
Blood Platelets
Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.
Sarcoma Viruses, Murine
A group of replication-defective viruses, in the genus GAMMARETROVIRUS, which are capable of transforming cells, but which replicate and produce tumors only in the presence of Murine leukemia viruses (LEUKEMIA VIRUS, MURINE).
Chromosome Aberrations
Abnormal number or structure of chromosomes. Chromosome aberrations may result in CHROMOSOME DISORDERS.
Receptors, Cytokine
Cell surface proteins that bind cytokines and trigger intracellular changes influencing the behavior of cells.
Erythropoietin
Glycoprotein hormone, secreted chiefly by the KIDNEY in the adult and the LIVER in the FETUS, that acts on erythroid stem cells of the BONE MARROW to stimulate proliferation and differentiation.
Antigens, CD34
Glycoproteins found on immature hematopoietic cells and endothelial cells. They are the only molecules to date whose expression within the blood system is restricted to a small number of progenitor cells in the bone marrow.
Cell Proliferation
All of the processes involved in increasing CELL NUMBER including CELL DIVISION.
Eosinophilia
Abnormal increase of EOSINOPHILS in the blood, tissues or organs.
Spleen
An encapsulated lymphatic organ through which venous blood filters.
Leukocyte Count
The number of WHITE BLOOD CELLS per unit volume in venous BLOOD. A differential leukocyte count measures the relative numbers of the different types of white cells.
Retroviridae
Family of RNA viruses that infects birds and mammals and encodes the enzyme reverse transcriptase. The family contains seven genera: DELTARETROVIRUS; LENTIVIRUS; RETROVIRUSES TYPE B, MAMMALIAN; ALPHARETROVIRUS; GAMMARETROVIRUS; RETROVIRUSES TYPE D; and SPUMAVIRUS. A key feature of retrovirus biology is the synthesis of a DNA copy of the genome which is integrated into cellular DNA. After integration it is sometimes not expressed but maintained in a latent state (PROVIRUSES).
Receptors, Erythropoietin
Cell surface proteins that bind erythropoietin with high affinity and trigger intracellular changes influencing the behavior of cells.
Chromosome Disorders
Clinical conditions caused by an abnormal chromosome constitution in which there is extra or missing chromosome material (either a whole chromosome or a chromosome segment). (from Thompson et al., Genetics in Medicine, 5th ed, p429)
Blood Cells
The cells found in the body fluid circulating throughout the CARDIOVASCULAR SYSTEM.
Genetic Testing
Detection of a MUTATION; GENOTYPE; KARYOTYPE; or specific ALLELES associated with genetic traits, heritable diseases, or predisposition to a disease, or that may lead to the disease in descendants. It includes prenatal genetic testing.
Moloney murine sarcoma virus
A replication-defective murine sarcoma virus (SARCOMA VIRUSES, MURINE) isolated from a rhabdomyosarcoma by Moloney in 1966.
Mice, Inbred C57BL
Mice, Transgenic
Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.
Receptor Protein-Tyrosine Kinases
A class of cellular receptors that have an intrinsic PROTEIN-TYROSINE KINASE activity.
Erythropoiesis
The production of red blood cells (ERYTHROCYTES). In humans, erythrocytes are produced by the YOLK SAC in the first trimester; by the liver in the second trimester; by the BONE MARROW in the third trimester and after birth. In normal individuals, the erythrocyte count in the peripheral blood remains relatively constant implying a balance between the rate of erythrocyte production and rate of destruction.
Base Sequence
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
Anemia, Refractory
A severe sometimes chronic anemia, usually macrocytic in type, that does not respond to ordinary antianemic therapy.
Protein Kinase Inhibitors
Agents that inhibit PROTEIN KINASES.
STAT5 Transcription Factor
A signal transducer and activator of transcription that mediates cellular responses to a variety of CYTOKINES. Stat5 activation is associated with transcription of CELL CYCLE regulators such as CYCLIN KINASE INHIBITOR P21 and anti-apoptotic genes such as BCL-2 GENES. Stat5 is constitutively activated in many patients with acute MYELOID LEUKEMIA.
Flow Cytometry
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
Disease Progression
The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.
Depressive Disorder, Major
Marked depression appearing in the involution period and characterized by hallucinations, delusions, paranoia, and agitation.

Mutant N-ras induces myeloproliferative disorders and apoptosis in bone marrow repopulated mice. (1/769)

Mutations that activate the N-ras oncogene are among the most frequently detected genetic alterations in human acute myeloid leukemias (AMLs), Philadelphia chromosome-negative myeloproliferative disorders (MPDs), and myelodysplastic syndromes (MDSs). However, because N-ras has not been shown to induce these disorders in an in vivo model, the role of N-ras in the evolution of myeloid leukemia is unclear. To investigate the potential of N-ras to induce myeloid leukemia, lethally irradiated mice were reconstituted with bone marrow (BM) cells infected with a retroviral vector carrying activated N-ras. Approximately 60% of these mice developed hematopoietic disorders, including severe MPDs resembling human chronic myelogenous leukemia (CML) or AML with differentiation (French-American-British [FAB] classification M2). Other reconstituted mice succumbed to hematopoietic defects that were pathologically similar to human MDSs. The latter disorders appeared to be due to a myeloid impairment that was demonstrated by enumeration of day-12 colony-forming units-spleen (CFU-S) and by in vitro colony assays. A high level of apoptosis associated with thymic atrophy and peripheral blood (PB) lymphopenia was also evident in N-ras reconstituted mice. Our results are consistent with a model in which antiproliferative effects are a primary consequence of N-ras mutations and secondary transforming events are necessary for the development of myeloid leukemia. This is the first report of an in vivo model for N-ras induced MPD and leukemia.  (+info)

Patients with thrombocytosis have normal or slightly elevated thrombopoietin levels. (2/769)

BACKGROUND AND OBJECTIVE: The distinction between clonal and reactive thrombocytoses is a frequent problem and implies different therapeutic options. As thrombopoietin (TPO) is the main regulator of megakaryocytopoiesis and thrombopoiesis, we measured TPO levels in patients with thrombocytosis in an attempt to understand the regulation and potential utility of distinguishing thrombocytoses. DESIGN AND METHODS: Serum TPO levels, platelet counts, mean platelet volume, hemoglobin, erythrocyte sedimentation rate and age were evaluated in 25 patients with clonal thrombocytosis (15 with essential thrombocythemia, 6 with polycythemia vera and 4 with chronic myeloid leukemia) and in 50 patients with reactive thrombocytosis distributed in three groups: 1) patients in post-surgical states; 2) patients with solid tumors; and 3) patients with inflammatory diseases. RESULTS: TPO levels were slightly increased in patients with clonal (135+/-50 pg/mL) and reactive (147+/-58 pg/mL) thrombocytosis compared with controls (121+/-58 pg/mL). Analyzing the different groups, patients with essential thrombocythemia had the lowest TPO levels (120+/-28 pg/mL) and patients with solid tumors the highest levels (162+/-59 pg/mL). Patients with clonal thrombocytosis were older, had higher platelet counts, mean platelet volume and hemoglobin, and lower erythrocyte sedimentation rate than patients with reactive thrombocytosis. INTERPRETATION AND CONCLUSIONS: Minor differences were observed in TPO levels between patients with primary and secondary thrombocytoses. Erythrocyte sedimentation rate, but not TPO levels, may be a useful tool for discriminating both types of thrombocytoses.  (+info)

Trisomy 21 associated transient neonatal myeloproliferation in the absence of Down's syndrome. (3/769)

Although usually associated with Down's syndrome, transient neonatal myeloproliferation (TMD) can occur in the absence of a constitutional trisomy 21. This report describes two such cases, both of whom had a trisomy 21 restricted to clonal cells. Unlike in previous such reported cases, spontaneous morphological, cytogenetic, and molecular remission in both cases was followed by re-emergence, in one case, of an evolved clone with a more malignant phenotype which required pharmacological intervention. Awareness that trisomy 21 bearing leukaemia in the neonatal period can be transient even in the absence of Down's syndrome is important to prevent unnecessary treatment. Equally, such cases require indefinite follow up as a proportion may have a recurrence which may require treatment.  (+info)

Characterization of FIM-FGFR1, the fusion product of the myeloproliferative disorder-associated t(8;13) translocation. (4/769)

The t(8;13) translocation found in a rare type of stem cell myeloproliferative disorder generates a constitutively activated tyrosine kinase containing N-terminal sequence encoded by the FIM gene linked to the FGFR1 kinase domain. Here we have further characterized FIM and FIM-FGFR1 proteins. Firstly, we have studied their respective subcellular localization. We show that FIM has nuclear and nucleolar localization, whereas FIM-FGFR1 is mainly cytoplasmic. Within the nucleolus, FIM colocalizes with the upstream binding factor in interphasic cells, indicating that FIM may be involved in the regulation of rRNA transcription. We demonstrate that the targetting of FIM to the nucleus depends upon its C-terminal region, which is absent in the cytoplasmic FIM-FGFR1 protein. Secondly, we demonstrate that FIM-FGFR1 has constitutive dimerization capability mediated by the FIM N-terminal sequences. Finally, we show that FIM-FGFR1 promotes survival of pro-B Ba/F3 cells after interleukin-3 withdrawal, whereas ligand-activated FGFR1 induced not only cell survival but also interleukin-3 independence. Taken together, these results indicate that FIM-FGFR1 is activated by dimerization as a cytoplasmic kinase and suggest that FIM-FGFR1 partially signals through the FGFR1 pathways.  (+info)

Bcr-Abl with an SH3 deletion retains the ability To induce a myeloproliferative disease in mice, yet c-Abl activated by an SH3 deletion induces only lymphoid malignancy. (5/769)

The bcr-abl oncogene plays a critical role in the pathogenesis of chronic myelogenous leukemia (CML). The fusion of Bcr sequences to Abl constitutively activates the Abl protein tyrosine kinase. We have recently shown that expression of Bcr-Abl in bone marrow cells by retroviral transduction efficiently induces in mice a myeloproliferative disease resembling human CML and that Abl kinase activity is essential for Bcr-Abl to induce a CML-like myeloproliferative disease. However, it is not known if activation of the Abl kinase alone is sufficient to induce a myeloproliferative disease. In this study, we examined the role of the Abl SH3 domain of Bcr-Abl in induction of myeloproliferative disease and tested whether c-Abl activated by SH3 deletion can induce a CML-like disease. We found that Bcr-Abl with an Abl SH3 deletion still induced a CML-like disease in mice. In contrast, c-Abl activated by SH3 deletion induced only lymphoid malignancies in mice and did not stimulate the growth of myeloid colonies from 5-fluorouracil-treated bone marrow cells in vitro. These results indicate that Bcr sequences in Bcr-Abl play additional roles in inducing myeloproliferative disease beyond simply activating the Abl kinase domain and that functions of the Abl SH3 domain are either not required or redundant in Bcr-Abl-induced myeloproliferative disease. The results also suggest that the type of hematological neoplasm induced by an abl oncogene is influenced not only by what type of hematopoietic cells the oncogene is targeted into but also by the intrinsic oncogenic properties of the particular abl oncogene. In addition, we found that DeltaSH3 c-Abl induced less activation of Akt and STAT5 than did Bcr-Abl, suggesting that activation of these pathways plays a critical role in inducing a CML-like disease.  (+info)

De novo appearance of the ph-1 chromosome in a previously monosomic bone marrow (45,XX,-6): conversion of a myeloproliferative disorder to acute myelogenous leukemia. (6/769)

Bone marrow examination of a patient with a myeloproliferative disorder revealed monosomy for chromosome No. 6 (45,XX,-6). Two months later, during blastic crisis, reinvestigation of the bone marrow showed the presence of the Ph-1 chromosome in the previously aneuploid cell line (45,XX,-6,-22,+Ph-1). This case differs from those previously published in that the Ph-1 chromosome appeared de novo during the development of frank acute myelogenous leukemia.  (+info)

Patients' psychosocial concerns following stem cell transplantation. (7/769)

Information regarding the nature, frequency, correlates and temporal trajectory of concerns of stem cell transplantation (SCT) recipients is critical to the development of interventions to enhance quality of life (QOL) in these individuals. This study examined psychosocial concerns in 110 SCT (87% autologous) recipients drawn from two SCT centers. Participants were a mean of 46 years of age and 17 months post-SCT (range 3-62 months). Information regarding current and past SCT-related concerns, performance status, and demographic characteristics was collected by telephone interview or questionnaire. Recipients reported a wide variety of psychosocial concerns following SCT. Recipients who were younger, female and evidenced a poorer performance status reported a larger number of post-SCT concerns. Examination of the temporal trajectory of concerns suggests that some concerns are salient throughout the course of post-SCT recovery (eg disease recurrence, energy level, 'returning to normal'), some are salient early in the course of recovery (eg quality of medical care, overprotectiveness by others), and others emerge later in the course of recovery (eg feeling tense or anxious, sexual life, sleep, relationship with spouse/partner, ability to be affectionate). Implications for the development of interventions to enhance post-SCT QOL are identified.  (+info)

Myeloproliferative disorders. (8/769)

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.  (+info)

Diagnostic refinement of chronic myeloproliferative disorders and thrombocytoses of unknown origin by multiple RT-PCR and...
TY - JOUR. T1 - Diagnostic refinement of chronic myeloproliferative disorders and thrombocytoses of unknown origin by multiple RT-PCR and capillary electrophoresis of BCR-ABL rearrangements and JAK2 (V617F) mutation. AU - Ammatuna, Emanuele. AU - Ottone, Tiziana. AU - Zaza, Serena. AU - Lavorgna, Serena. AU - Grillo, Rosa. AU - Curzi, Paola. AU - Panetta, Paola. AU - Federici, Giorgio. AU - Amadori, Sergio. AU - Lo-Coco, Francesco. PY - 2007/5. Y1 - 2007/5. N2 - Detection of genetic markers improves diagnostic refinement of chronic myeloproliferative disorders (CMDs) and is helpful in discriminating reactive conditions mimicking CMDs such as reactive erythrocytosis and thrombocytosis. We set-up a multiplex real-time polymerase chain reaction assay followed by capillary electrophoresis, designed to simultaneously screen the two main genetic lesions associated with CMDs, i.e. the BCR-ABL fusion characteristic of chronic myeloid leukemia and the JAK2 V617F mutation that characterises polycythaemia ...
https://moh-it.pure.elsevier.com/en/publications/diagnostic-refinement-of-chronic-myeloproliferative-disorders-and
Prenatal diagnosis of a transient myeloproliferative disorder in trisomy 21<...
TY - JOUR. T1 - Prenatal diagnosis of a transient myeloproliferative disorder in trisomy 21. AU - Baschat, A. A.. AU - Wagner, T.. AU - Malisius, R.. AU - Gembruch, U.. PY - 1998/7/1. Y1 - 1998/7/1. N2 - We report the prenatal diagnosis of a transient myeloproliferative disorder suggestive of leukaemia in a fetus with hepatosplenomegaly, hydrops and 47,XY,+21 karyotype. The initial fetal white blood cell count at 26 + 5 weeks gestation was 190/nl with 70 per cent blast cells. Immunophenotyping of the large blasts revealed surface markers suggestive of an early stem cell differentiation arrest resulting in undifferentiated polyclonal myelopoiesis. The fetal heart tracing showed minimal beat-to-beat variability in the presence of high leukocyte counts. Serial fetal blood sampling showed decreasing blast cells in the peripheral blood and normalization of white blood cell counts. Although there was increasing hydrops, this period was marked by improvement of the fetal heart rate pattern. Finally ...
https://jhu.pure.elsevier.com/en/publications/prenatal-diagnosis-of-a-transient-myeloproliferative-disorder-in--3
JAK2, CALR, and MPL Mutation Profiles in BCR-ABL Negative Myeloproliferative Neoplasms, a Referral Center Experience in the...JAK2, CALR, and MPL Mutation Profiles in BCR-ABL Negative Myeloproliferative Neoplasms, a Referral Center Experience in the...
Background: This study was conducted to evaluate the frequency of JAK2, CALR and MPL mutations in with BCR-ABL myeloproliferative neoplasms and their association with demographic data and hematologic parameters in a referral center, in the Middle East. Methods: Seventy-one patients with BCR-ABL negative myeloproliferative neoplasms were evaluated for JAK2 V617F, CALR type 1, type 2, and MPL by allele-specific PCR and conventional PCR from 2018 to 2019. Results: Twenty three patients were categorized as polycythemia vera and demonstrated JAK2 V617F in 91.3 % of these cases. Thirty-eight patients were classified as essential thrombocythemia and showed JAK2 V617F in 52.6%, CALR type 1 in 18.4%, CALR type 2 in 7.9% and no mutation in 21.1%. Seven patients were recognized as primary myelofibrosis and exhibited JAK2 V617F mutation in 57.1%, CALR type 1 in 14.3 %, CALR type 2 in 14.3% and no mutation in 14.3%. Three patients were diagnosed as MPN, unclassifiable and revealed JAK2 V617F mutation in 33.3% and
http://ijp.iranpath.org/article_241840.html
Screening for Pulmonary Vascular Changes in Patients With Chronic Myeloproliferative Diseases - Full Text View - ClinicalTrials...Screening for Pulmonary Vascular Changes in Patients With Chronic Myeloproliferative Diseases - Full Text View - ClinicalTrials...
Previous small studies and clinical cases have suggested a possible association between pulmonary hypertension (PH) and chronic myeloproliferative disorders (CMPD). MPD may cause PH through different mechanisms as: high cardiac output, asplenia, direct obstruction of pulmonary arteries by megakaryocytes, chronic thromboembolic endothelial pulmonary hypertension (CTEPH), porto-pulmonary hypertension (POPH). However, the exact prevalence of PH in this group of disorders is not known.. This study is designed to identify the pulmonary vascular changes and describe the prevalence of pulmonary hypertension (defined in this study as mean pulmonary arterial hypertension (mPAP) ≥25mmHg as assessed by right-heart catheterization (RHC) or systolic pulmonary arterial pressure (sPAP) ≥37mmHg (2.9 m/s) assessed by echocardiography. ...
https://clinicaltrials.gov/ct2/show/NCT01787162?term=%22High+Blood+Pressure%22&lup_s=01%2F26%2F2013&lup_d=14&show_rss=Y&sel_rss=mod14
Chronic Myeloproliferative Disorders
Chronic myeloproliferative disorders are a group of slow-growing blood cancers in which the bone marrow makes too many abnormal red blood cells, white blood cells, or platelets, which accumulate in the blood. The type of myeloproliferative disorder is based on whether too many red blood cells, white blood cells, or platelets are being made. Sometimes the body will make too many of more than one type of blood cell, but usually one type of blood cell is affected more than the others are ...
http://diseaseinfosearch.org/Chronic+Myeloproliferative+Disorders/1624
Clinical significance of neutrophil CD177 mRNA expression in Ph-negative chronic myeloproliferative disorders | IRIS UNIPVClinical significance of neutrophil CD177 mRNA expression in Ph-negative chronic myeloproliferative disorders | IRIS UNIPV
The PRV-1 gene has been proposed as a marker of polycythaemia vera (PV). PRV-1 and NB1 are alleles of the polymorphic gene CD177, which belongs to the Ly-6/uPAR superfamily, and their coding regions differ at only four nucleotides. We studied neutrophil CD177 mRNA levels in normal subjects and in 235 patients with Ph-negative chronic myeloproliferative disorders (CMD), including PV, essential thrombocythaemia and myelofibrosis with myeloid metaplasia. Additional disease states were investigated for comparison. Highly variable neutrophil CD177 mRNA levels were observed in normal individuals. Neutrophils isolated from the bone marrow, or from peripheral blood following granulocyte colony-stimulating factor administration showed markedly higher CD177 expression than circulating granulocytes on steady state. Increased neutrophil CD177 mRNA levels were detected in all CMD. Elevated values were also found in reactive conditions and in disorders such as chronic myeloid leukaemia and myelodysplastic ...
https://iris.unipv.it/handle/11571/115818
Janus kinase 2 mutations in Philadelphia negative chronic myeloproliferative disorders: clinical implications. - Semantic...Janus kinase 2 mutations in Philadelphia negative chronic myeloproliferative disorders: clinical implications. - Semantic...
The chronic myeloproliferative disorders (CMPD) are a group of clinically related diseases characterized by clonal hematopoiesis with increased proliferation of one or more myeloid cell lineages. The identification of JAK2 mutations (JAK2V617F and JAK2 exon 12) in patients with CMPD is of great significance in the understanding of the molecular mechanisms underlined the pathogenesis of the disease contributing also to clinical management of patients. However, the precise pathogenetic contribution of JAK2 mutation is far from being fully elucidated and it is currently under intense investigation. Testing of JAK2 mutations has made the diagnosis of CMPD more precise than ever before, while genotype-phenotype associations have been identified. Furthermore, the discovery of JAK2 mutations facilitated the development of new targeted therapies and clinical trials are currently ongoing.
https://www.semanticscholar.org/paper/Janus-kinase-2-mutations-in-Philadelphia-negative-Panani/659dc7b5492c5a43889fc80791edfb927893223d
Inflammatory picture of Philadelphia-negative myeloproliferative neoplasms | Hematology, Transfusion and Cell Therapy
In this issue of the Hematology, Transfusion and Cell Therapy Journal, Cacemiro et al. evaluated the plasma cytokine profile of 47 patients with Ph-negative myeloproliferative neoplasms (MPN) [essential thrombocythemia (ET), primary myelofibrosis (PMF), and polycythemia vera (PV)] and of healthy subjects.1 They demonstrated increased levels of pro-inflammatory cytokines in MPN patients and higher levels of interferon (IFN)-γ-induced protein 10 (IP-10) in PMF patients with the JAK2 V617F mutation. They found differences in the cytokine profile among the three MPN disorders, including increased levels of IL-12p70, IL-17A, and RANTES in PMF, showing that MPN, in particular PMF, have altered inflammatory profiles. However, their sample population did not make clinical and prognostic implications of their findings possible.. What is the clinical relevance of the altered cytokine levels in MPN? Are they related to constitutional symptoms, transformation or evolution to fibrosis? Do they have an ...
http://www.htct.com.br/en-inflammatory-picture-philadelphia-negative-myeloproliferative-neoplasms-articulo-S253113791830052X
Analysis of GATA1 mutations in Down syndrome transient myeloproliferative disorder and myeloid leukemia. - Department of...
Children with Down syndrome (DS) up to the age of 4 years are at a 150-fold excess risk of developing myeloid leukemia (ML-DS). Approximately 4%-5% of newborns with DS develop transient myeloproliferative disorder (TMD). Blast cell structure and immunophenotype are similar in TMD and ML-DS. A mutation in the hematopoietic transcription factor GATA1 is present in almost all cases. Here, we show that simple techniques detect GATA1 mutations in the largest series of TMD (n = 134; 88%) and ML-DS (n = 103; 85%) cases tested. Furthermore, no significant difference in the mutational spectrum between the 2 disorders was seen. Thus, the type of GATA1 sequence mutation is not a reliable tool and is not prognostic of which patients with TMD are probable to develop ML-DS.
https://www.paediatrics.ox.ac.uk/publications/163951
Myeloproliferative neoplasm - WikipediaMyeloproliferative neoplasm - Wikipedia
The myeloproliferative neoplasms (MPNs), previously myeloproliferative diseases (MPDs), are a group of diseases of the bone marrow in which excess cells are produced. They are related to, and may evolve into, myelodysplastic syndrome and acute myeloid leukemia, although the myeloproliferative diseases on the whole have a much better prognosis than these conditions. The concept of myeloproliferative disease was first proposed in 1951 by the hematologist William Dameshek.[1] In the most recent World Health Organization classification of hematologic malignancies, this group of diseases was renamed from myeloproliferative diseases to myeloproliferative neoplasms.[2] This reflects the underlying clonal genetic changes that are a salient feature of this group of disease.. The increased numbers of blood cells may not cause any symptoms, but a number of medical problems or symptoms may occur. The risk of thrombosis is increased in some types of MPN.. ...
https://en.wikipedia.org/wiki/Myeloproliferative_disease
8p11 myeloproliferative syndrome: diagnostic challenges and pitfalls. | PubFacts8p11 myeloproliferative syndrome: diagnostic challenges and pitfalls. | PubFacts
8p11 myeloproliferative syndrome (EMS) is a very rare clinicopathological entity which is characterized by the appearance of a myeloproliferative neoplasm in the bone marrow, peripheral lymphadenopathy, usually caused by T or B lymphoblastic lymphoma/leukemia, and a reciprocal translocation involving chromosome 8p11. Herein we describe a 22-year-old male patient with unusual clinical presentation of EMS. Namely, he initially presented with prolonged epistaxis. Complete blood count showed elevated hemoglobin (17.7g/dl), thrombocytopenia (98x109/l) and leukocytosis (57x109/l). Bone marrow aspirate and biopsy findings corresponded with the presence of a myeloproliferative neoplasm while cytogenetic analysis revealed t(8;13)(p11q12). After that ZMYM2-FGFR1 in-frame fusion was confirmed at the molecular level. Immediately after establishing the diagnosis of a myeloproliferative neoplasm (MPN) generalized lymphadenopathy was developed. Histopathologic examination of lymph node sample confirmed the ...
https://www.pubfacts.com/detail/27569099/8p11-myeloproliferative-syndrome-diagnostic-challenges-and-pitfalls
Acute progression of BCR-FGFR1 induced murine B-lympho/myeloproliferative disorder suggests involvement of lineages at the pro...
TY - JOUR. T1 - Acute progression of BCR-FGFR1 induced murine B-lympho/myeloproliferative disorder suggests involvement of lineages at the pro-B cell stage. AU - Ren, Mingqiang. AU - Tidwell, Josephine A.. AU - Sharma, Suash. AU - Cowell, John Kenneth. PY - 2012/6/6. Y1 - 2012/6/6. N2 - Constitutive activation of FGFR1, through rearrangement with various dimerization domains, leads to atypical myeloproliferative disorders where, although T cell lymphoma are common, the BCR-FGFR1 chimeric kinase results in CML-like leukemia. As with the human disease, mouse bone marrow transduction/transplantation with BCR-FGFR1 leads to CML-like myeloproliferation as well as B-cell leukemia/lymphoma. The murine disease described in this report is virtually identical to the human disease in that both showed bi-lineage involvement of myeloid and B-cells, splenomegaly, leukocytosis and bone marrow hypercellularity. A CD19+ IgM- CD43+ immunophenotype was seen both in primary tumors and two cell lines derived from ...
https://augusta.pure.elsevier.com/en/publications/acute-progression-of-bcr-fgfr1-induced-murine-b-lymphomyeloprolif
The mutation profile of JAK2 and CALR in Chinese Han patients with Philadelphia chromosome-negative myeloproliferative...The mutation profile of JAK2 and CALR in Chinese Han patients with Philadelphia chromosome-negative myeloproliferative...
Mutations in JAK2, MPL and CALR are highly relevant to the Philadelphia chromosome (Ph)-negative myeloproliferative neoplasms (MPNs). We performed high resolution melting analysis and Sanger sequencing together with T-A cloning to elucidate the unique mutation profile of these genes, in Chinese patients with MPNs. Peripheral blood DNA samples were obtained from 80 patients with polycythemia vera (PV), 80 patients with essential thrombocytosis (ET) and 50 patients with primary myelofibrosis (PMF). Ten PV patients were identified with diverse JAK2 exon 12 mutations. Five novel JAK2 Exon 12 mutation patterns (M532V/E543G, N533D, M535I/H538Y/K549I, E543G and D544N) were described. JAK2 V617F was detected in 140 samples (66 PV, 45 ET and 29 PMF). JAK2 Exon 12 mutations were prevalent (13%) and variable in the Chinese patients. Compared with PV patients with JAK2 V617F mutations, PV patients with JAK2 exon 12 mutations had an earlier median onset of disease (P = 0.0013). MPL W515L/K mutations were ...
https://read.qxmd.com/read/25023898/the-mutation-profile-of-jak2-and-calr-in-chinese-han-patients-with-philadelphia-chromosome-negative-myeloproliferative-neoplasms
Complementary and Alternative Medicine - Myeloproliferative disordersComplementary and Alternative Medicine - Myeloproliferative disorders
Abraham S, Salama M, Hancock J, Jacobsen J, Fluchel M. Congenital and childhood myeloproliferative disorders with eosinophilia responsive to imatinib. Pediatr Blood Cancer. 2012;59(5):928-9.. Bauer JD, Capra S. Nutrition intervention improves outcomes in patients with cancer cachexia receiving chemotherapy -- a pilot study. Support Care Cancer. 2005;13(4):270-4.. Bell DR, Gochenaur K. Direct vasoactive and vasoprotective properties of anthocyanin-rich extracts. J Appl Physiol. 2006;100(4):1164-70.. Bruchova H, Merkerova M, Prchal JT. Aberrant expression of microRNA in polycythemia vera. Haematologica. 2008;93(7):1009-16.. Cabrera C, Artacho R, Gimenez R. Beneficial effects of green tea--a review. J Am Coll Nutr. 2006;25(2):79-99.. Chen F, Li L, Ma D, et al. Imatinib achieved complete cytogenetic response in a CML patient received 32-year indirubin and its derivative treatment. Leuk Res. 2010 Feb;34(2):e75-7.. de Lacerda JF, Oliveira SN, Ferro JM. Chronic myeloproliferative diseases. Handb Clin ...
http://owensborohealthse3.adam.com/content.aspx?productid=107&isarticlelink=false&pid=33&gid=000114
Complications of Myelodysplastic/myeloproliferative disease - RightDiagnosis.comComplications of Myelodysplastic/myeloproliferative disease - RightDiagnosis.com
Complications of Myelodysplastic/myeloproliferative disease including hidden complications, secondary medical conditions, symptoms, or other types of Myelodysplastic/myeloproliferative disease complication.
http://www.rightdiagnosis.com/m/myelodysplastic_myeloproliferative_disease/complic.htm
Magnitude of Thrombosis Risk in Patients With Myeloproliferative Neoplasms | Annals of Internal Medicine | American College of...Magnitude of Thrombosis Risk in Patients With Myeloproliferative Neoplasms | Annals of Internal Medicine | American College of...
Myeloproliferative diseases were first described by William Dameshek in 1951. In 2008, the World Health Organization established a new classification system and introduced the term myeloproliferative neoplasms (MPNs). Polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are the most prevalent MPNs and are characterized by overproduction of leukocytes, erythrocytes, or platelets; development of bone marrow fibrosis; leukemic transformation; and arterial and venous thrombosis. When Dameshek proposed the term myeloproliferative diseases, he also proposed the presence of a then-undiscovered stimulus that drove proliferation. We now understand that mutation of the JAK2 gene, JAK2 V617F, is the most common stimulus, occurring in 95% of patients with PV and 60% of those with ET or PMF. Myeloproliferative neoplasms are relatively rare; are acquired in middle to older age; and are, despite their classification as neoplasms, indolent diseases, with survival measured ...
http://annals.org/aim/article-abstract/2670030/shedding-new-light-magnitude-thrombosis-risk-patients-myeloproliferative-neoplasms
Philadelphia-negative myeloproliferative neoplasms as disorders marked by cytokine modulationPhiladelphia-negative myeloproliferative neoplasms as disorders marked by cytokine modulation
Conclusions:. Essential thrombocythemia, primary myelofibrosis, and polycythemia vera patients exhibited different patterns of cytokine production, as revealed by cytokine network correlations. Together, these findings suggest that augmented cytokine levels are associated with the physiopathology of myeloproliferative neoplasms. ...
http://www.scielo.br/scielo.php?script=sci_abstract&pid=S2531-13792018000200120&lng=pt&nrm=iso
Risk Factors and Predictors of Thrombosis in Myeloprolferative Neoplasms - Full Text View - ClinicalTrials.govRisk Factors and Predictors of Thrombosis in Myeloprolferative Neoplasms - Full Text View - ClinicalTrials.gov
Classic BCR-ABL negative chronic myeloproliferative neoplasms (MPN) are stem cell disorders characterized by abnormal myeloid proliferation and increased blood cell counts and comprise polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF).. Myeloproliferative neoplasms (MPN) are common malignancies in elderly individuals as the combined annual incidence rates of classical MPNs were reported to be 0.84, 1.03, and 0.47 per 100,000 respectively for PV, ET, and PMF but with a wide variation in prevalence rates reported in different studies. The 5-year relative survival rates are 84.8, 89.9, and 39% for PV, ET and PMF patients, respectively.. Thromboembolic complications represent a major cause of morbidity and mortality in MPN, particularly in PV and ET. The mechanisms underlying increased thrombotic risk in chronic myeloproliferative neoplasms (MPN) are incompletely understood.. Several pathophysiological mechanisms help explain the increased likelihood of ...
https://clinicaltrials.gov/ct2/show/NCT03599700
Practical management of classical myeloproliferative disorder patients: A clinicians guide<...
TY - JOUR. T1 - Practical management of classical myeloproliferative disorder patients. T2 - A clinicians guide. AU - Mesa, Ruben A.. PY - 2006/8/1. Y1 - 2006/8/1. N2 - The classical myeloproliferative disorders (MPDs) are comprised of the clonal, BCR-ABL-negative, chronic myelold disorders of essential thrombocythemia, polycythemia vera, and myelofibrosis with myeloid metaplasia. Managment of these disorders remains a significant challenge due to the varied range of prognosis and phenotypic manifestations. Curative therapy, achieved in some patients through allogeneic stem cell transplantation, is elusive or inappropriate in most. Additionally, no available medical therapy has been shown to clearly improve survival or delay disease progression. Current management involves an emphasis on prevention of thrombohemorrhagic complications (through aspirin treatment, phlebotomy and cytoreduction in high-risk patients) in early-stage patients and symptomatic care in those with advanced disease. ...
https://mayoclinic.pure.elsevier.com/en/publications/practical-management-of-classical-myeloproliferative-disorder-pat
Myeloproliferative Neoplasms | Leukemia and Lymphoma SocietyMyeloproliferative Neoplasms | Leukemia and Lymphoma Society
Myeloproliferative Neoplasms (MPNs): Diagnosis, Treatment and Side Effects Management This continuing education virtual lecture on myeloproliferative neoplasms diagnosis, treatment and side effects management is for nurses, nurse practitioners, and oncology social workers. Topics covered include types of myeloproliferative neoplasms tests for diagnosis, treatments and management of side effects.The material is presented by a physician, a pharmacist and a nurse practitioner. There is no fee for this educational activity.
http://www.lls.org/professional-education-webcasts/myeloproliferative-neoplasms-for-nurses?src1=22546&src2=
Myeloproliferative NeoplasmsMyeloproliferative Neoplasms
Know more about the symptoms, causes, diagnosis and treatment for Myeloproliferative Neoplasms. mfine has the finest of Oncologist who will provide the best treatment.
https://www.mfine.co/oncologists/conditions/myeloproliferative-neoplasms/
Normal hematopoiesis and neurofibromin-deficient myeloproliferative disease require Erk<...
TY - JOUR. T1 - Normal hematopoiesis and neurofibromin-deficient myeloproliferative disease require Erk. AU - Staser, Karl. AU - Park, Su Jung. AU - Rhodes, Steven D.. AU - Zeng, Yi. AU - He, Yong Zheng. AU - Shew, Matthew A.. AU - Gehlhausen, Jeffrey R.. AU - Cerabona, Donna. AU - Menon, Keshav. AU - Chen, Shi. AU - Sun, Zejin. AU - Yuan, Jin. AU - Ingram, David A.. AU - Nalepa, Grzegorz. AU - Yang, Feng Chun. AU - Clapp, D. Wade. PY - 2013/1/2. Y1 - 2013/1/2. N2 - Neurofibromatosis type 1 (NF1) predisposes individuals to the development of juvenile myelomonocytic leukemia (JMML), a fatal myeloproliferative disease (MPD). In genetically engineered murine models, nullizygosity of Nf1, a tumor suppressor gene that encodes a Ras-GTPase-activating protein, results in hyperactivity of Raf/Mek/Erk in hematopoietic stem and progenitor cells (HSPCs). Activated Erk1/2 phosphorylate kinases and transcription factors with myriad mitogenic roles in diverse cell types. However, genetic studies examining ...
https://miami.pure.elsevier.com/en/publications/normal-hematopoiesis-and-neurofibromin-deficient-myeloproliferati
8p11 myeloproliferative syndrome: a review8p11 myeloproliferative syndrome: a review
The 8p11 myeloproliferative syndrome is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 tyrosine kinase gene on chromosome 8p11-12. By our count, 65 cases are currently reported in the literature. This neoplasm affects patients of a …
https://pubmed.ncbi.nlm.nih.gov/20226962/?dopt=Abstract
CHROMOSOME 8p11 MYELOPROLIFERATIVE SYNDROME | MENDELIAN.COCHROMOSOME 8p11 MYELOPROLIFERATIVE SYNDROME | MENDELIAN.CO
CHROMOSOME 8p11 MYELOPROLIFERATIVE SYNDROME description, symptoms and related genes. Get the complete information in our medical search engine for phe
https://www.mendelian.co/diseases/chromosome-8p11-myeloproliferative-syndrome
Modified PCR-RFLP for detection of JAK2V617F mutation in patients with myeloproliferative neoplasm - Scientific Journal of...Modified PCR-RFLP for detection of JAK2V617F mutation in patients with myeloproliferative neoplasm - Scientific Journal of...
Abstract Background and Objectives Chronic myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell disorders. They are heterogeneous in symptoms and mainly consist of Philadelphia chromosome positive (Ph+) and negative (Ph-). The Ph- group includes polycythemia Vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and other rare disorders. In the latter ...
http://bloodjournal.ir/article-1-1093-en.html
The effects of mutational profiles on phenotypic presentation of myeloproliferative neoplasm subtypes in Bosnia: 18 year follow...The effects of mutational profiles on phenotypic presentation of myeloproliferative neoplasm subtypes in Bosnia: 18 year follow...
The identification of mutually exclusive somatic mutations shared among myeloproliferative neoplasm (MPN) subtypes has provided a powerful tool for studying disease evolution. Clinical features, gene mutations, and survival over 18 years were analyzed in MPN patients. One hundred thirty-eight MPN patients were subcategorized according to MPN subtypes: essential thrombocythemia (ET, n = 41), polycythemia vera (PV, n = 56), primary myelofibrosis (PMF, n = 10), and MPN unclassified (MPN-U, n = 31). Patient characteristics included clinical parameters, overall survival (OS), and mutational status of the Janus kinase 2 (JAK2), calreticulin (CALR), and myeloproliferative leukemia virus oncogene (MPL) genes. We compared hematologic and clinical features of JAK2V617F-ET vs. CALR-mutated ET vs. JAK2V617F-PV patients. JAK2V617F-patients had higher values of erythrocytes, hemoglobin, and hematocrit compared to CALR-mutated patients (p , 0.05). The mutant allele burden in JAK2V617F-PV and JAK2V617F-ET ...
https://www.bjbms.org/ojs/index.php/bjbms/article/view/4391
Chronic myeloproliferative disorders with myeloid metaplasia | AVESİS
Copy For Citation Kabukcuoolu S. AMERICAN JOURNAL OF SURGICAL PATHOLOGY, vol.24, no.10, pp.1437, 2000 (Journal Indexed in SCI) ...
https://avesis.ogu.edu.tr/yayin/9069aec6-8557-426d-9183-783a5851afd9/chronic-myeloproliferative-disorders-with-myeloid-metaplasia
Long-term Study Evaluating the Effect of Givinostat in Patients With JAK2V617F Positive Chronic Myeloproliferative Neoplasms -...Long-term Study Evaluating the Effect of Givinostat in Patients With JAK2V617F Positive Chronic Myeloproliferative Neoplasms -...
This study is investigating the efficacy and tolerability of givinostat [Italfarmaco] in the treatment of patients with JAK2V617F positive, chronic
http://adisinsight.springer.com/trials/700227911?error=cookies_not_supported&code=a0d903ae-f87b-41e9-a31d-7dc7c72a58ce
Digitum: Repositorio Institucional de la Universidad de Murcia: Myelofibrosis in chronic myeloproliferative disorders -...Digitum: Repositorio Institucional de la Universidad de Murcia: Myelofibrosis in chronic myeloproliferative disorders -...
DIGITUM es el Repositorio Institucional de la Universidad de Murcia. Su objetivo es permitir el acceso libre a la producción científica y académica de la Universidad.
https://digitum.um.es/digitum/handle/10201/22602
Multicenter Retrospective Analysis of Turkish Patients with Chronic Myeloproliferative Neoplasms [Turk J Hematol]
Bulgular: JAK-2 mutasyonu PV li hastalar n %86 s nda, ET li hastalar n %51,5 inde ve PMF li hastalar n %50,4 nde pozitif bulundu. Tan da tromboz ve kanama, PV li hastalar n s ras yla %20,6 ve %7,5 inde, ET li hastalar n %15,1 ve %9 unda ve PMF li hastalar n %9,5 ve %10,4 nde saptand . Alt y z sekiz hasta (%85,9) sitored ktif tedavi alm t . En s k kullan lan ila hidroksi re (%89,6) idi. L semik ve fibrotik transformasyon s kl %0,6 ve %13,2 idi. 10 y ll k hesaplanan toplam sa kal m PV, ET ve PMF hastalar nda s ras yla %89,7, %85 ve %82,5 idi. 10 y ll k toplam sa kal m a s ndan ET, PV ve PMF hastalar nda anlaml fark yoktu ...
http://aoh.tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-54280
Clinical Features of 294 Turkish Patients with Chronic Myeloproliferative Neoplasms [Turk J Hematol]
Bulgular: leri ya , tan da y ksek l kosit say s JAK2 mutasyon pozitifli i tromboz i in risk fakt r olarak bulunmu tur. Trombosit say m n n 1000x109/L zerinde olmas kanama komplikasyonlar a s ndan risk fakt r d r. Hidroksi re tedavisi l semik d n mle ili kili bulunmam t r. Bu hastalarda: Medyan takip s resi 50 ay (22,2- 81,75 eyrekler) idi. Primer miyelofibrozisli hastalar ET i in 179 ay ve PV i in 231 ay olan ya am s releri ile kar la t r ld nda 137 ay ile en k sa ya am s resine sahip hastalard r. L semik transformasyon, tromboembolik olaylar, 60 ya st olmak ve anemi ya am s resinin etkileyen fakt rler olarak bulunmu tur ...
http://aoh.tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-97992
Chronic Myeloproliferative Neoplasms Treatment (Fact Sheet) - Oncology Nurse Advisor - Global GenesChronic Myeloproliferative Neoplasms Treatment (Fact Sheet) - Oncology Nurse Advisor - Global Genes
Please join the RARE Portal to add diseases of interest to your personal profile. By creating a profile, you can receive news, resources and updates related to this disease as well as many other benefits. ...
https://globalgenes.org/news/chronic-myeloproliferative-neoplasms-treatment-fact-sheet-oncology-nurse-advisor/
Diagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders | HaematologicaDiagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders | Haematologica
Following the success of imatinib in BCR-ABL positive CML and its capacity to occupy the ATP binding sites of several other tyrosine kinases such as ARG (ABL2), KIT, PDGFRA, PDGFRB or FMS, there has been considerable interest in extending its clinical use to diseases in which other activated tyrosine kinases are implicated. In this issue Baccarani et al.27 present the results of a phase-II-trial in which patients with FIP1L1-PDGFRA-positive CEL or HES without a known molecular target were treated with imatinib at a target dose of 400 mg/day. This is the largest such study reported to date and has the advantage of relatively long follow-up. Based on the high rates of rapid complete hematologic remissions previously reported by other groups,27 it is not surprising that all FIP1L1-PDGFRA positive patients achieved complete hematologic remissions after a median of 2 months. In addition, all patients achieved complete molecular remission, as determined by nested RT-PCR, after a median of 6 months, ...
http://www.haematologica.org/content/92/9/1153
Philadelphia negative myeloproliferative neoplasms | OncologyPROPhiladelphia negative myeloproliferative neoplasms | OncologyPRO
ESMO is a Swiss-registered not-for-profit organisation. All funding for this site is provided directly by ESMO or via grants from the sponsors and supporters.. Via L. Taddei 4, 6962 Viganello - Lugano - CH © Copyright 2017 European Society for Medical Oncology All rights reserved worldwide.. ...
http://oncologypro.esmo.org/Topics/Anti-Cancer-Agents-Biologic-Therapy/Philadelphia-negative-myeloproliferative-neoplasms
Methylome profiling reveals distinct alterations in phenotypic and mutational subgroups of myeloproliferative neoplasms<...
TY - JOUR. T1 - Methylome profiling reveals distinct alterations in phenotypic and mutational subgroups of myeloproliferative neoplasms. AU - Nischal, Sangeeta. AU - Bhattacharyya, Sanchari. AU - Christopeit, Maximilian. AU - Yu, Yiting. AU - Zhou, Li. AU - Bhagat, Tushar D.. AU - Sohal, Davendra. AU - Will, Britta. AU - Mo, Yongkai. AU - Suzuki, Masako. AU - Pardanani, Animesh. AU - Michael McDevitt, McDevitt. AU - Maciejewski, Jaroslaw P.. AU - Melnick, Ari M.. AU - Greally, John M.. AU - Steidl, Ulrich. AU - Moliterno, Alison R. AU - Verma, Amit. PY - 2013/2/1. Y1 - 2013/2/1. N2 - Even though mutations in epigenetic regulators frequently occur in myeloproliferative neoplasms, their effects on the epigenome have not been well studied. Furthermore, even though primary myelofibrosis (PMF) has a markedly worse prognosis than essential thrombocytosis or polycythemia vera, the molecular distinctions between these subgroups are not well elucidated. We conducted the HELP (HpaII tiny fragment enriched ...
https://jhu.pure.elsevier.com/en/publications/methylome-profiling-reveals-distinct-alterations-in-phenotypic-an-6
Mutations with Epigenetic Effects in Myeloproliferative Neoplasms and Recent Progress in Treatment: Proceedings from the 5th...Mutations with Epigenetic Effects in Myeloproliferative Neoplasms and Recent Progress in Treatment: Proceedings from the 5th...
Immediately following the 2010 annual American Society of Hematology (ASH) meeting, the 5th International Post-ASH Symposium on Chronic Myelogenous Leukemia and BCR-ABL1-Negative Myeloproliferative Neoplasms (MPNs) took place on 7-8 December 2010 in Orlando, Florida, USA. During this meeting, the most recent advances in laboratory research and clinical practice, including those that were presented at the 2010 ASH meeting, were discussed among recognized authorities in the field. The current paper summarizes the proceedings of this meeting in BCR-ABL1-negative MPN. We provide a detailed overview of new mutations with putative epigenetic effects (TET oncogene family member 2 (TET2), additional sex comb-like 1 (ASXL1), isocitrate dehydrogenase (IDH) and enhancer of zeste homolog 2 (EZH2)) and an update on treatment with Janus kinase (JAK) inhibitors, pomalidomide, everolimus, interferon-α, midostaurin and cladribine. In addition, the new Dynamic International Prognostic Scoring System ...
https://dash.harvard.edu/handle/1/8603493?show=full
Molecular diagnosis of myeloproliferative neoplasms<...
TY - JOUR. T1 - Molecular diagnosis of myeloproliferative neoplasms. AU - Patnaik, Mrinal M.. AU - Tefferi, Ayalew. PY - 2009/7/1. Y1 - 2009/7/1. N2 - The molecular profiling of myeloproliferative neoplasms (MPNs) has introduced a paradigm shift in the process of diagnosis, prognostication, monitoring and treatment of these diseases. The discovery of the BCR-ABL fusion oncogene is an example of a remarkable bench-to-bedside story. It has provided a comprehensive explanation of the pathogenesis of chronic myelogenous leukemia, and has resulted in the development of excellent treatment strategies. It has led to the use of advanced diagnostic techniques, such as fluorescence in situ hybridization and PCRs that allow for more effective means to monitor disease treatment, including the detection of minimal residual disease, early relapse and drug resistance. Unlike chronic myelogenous leukemia, the exact molecular pathways for the BCR-ABL-negative MPNs have not been completely elucidated. The ...
https://mayoclinic.pure.elsevier.com/en/publications/molecular-diagnosis-of-myeloproliferative-neoplasms
Managing Myeloproliferative Neoplasms : A Case-Based ApproachManaging Myeloproliferative Neoplasms : A Case-Based Approach
This is a concise, practical, case-based book documenting examples and scenarios that will help you manage challenging clinical issues for patients with myeloproliferative neoplasms. The editors and authors have strived to distil the very latest information in this rapidly advancing field in a way that will help you to update your practice and manage your patients. The key focuses are: diagnosis, both standard and challenging; both day-to-day management as well as special situations such as surgery, thrombotic events and pregnancy; and finally, managing evolving situations with MPN such as progression to acute myeloid leukemia. This book is an outstanding resource that includes a discussion of both classical myeloproliferative neoplasms, such as essential thrombocythemia, polycythemia vera and myelofibrosis, and also less common disorders such as systemic mast cell disease, hypereosinophilia, MPN/MBS overlap syndromes and atypical CML, amongst others. This book is a practical reference for ...
http://ebooksmedicine.net/managing-myeloproliferative-neoplasms-a-case-based-approach4540
Coexistence of lymphoproliferative and myeloproliferative neoplasms with simultaneous CALR and JAK2 V617F mutations. - Semantic...Coexistence of lymphoproliferative and myeloproliferative neoplasms with simultaneous CALR and JAK2 V617F mutations. - Semantic...
BACKGROUND Hematopoietic malignancies are a group of blood cell disorders characterized by abnormal hematopoietic proliferation. OBJECTIVE The identification of specific clinicopathologic characteristics and tumor-related gene status provides critical information on potential therapeutic targets. METHODS The specimens were tested with immunohistochemistry, flow cytometry, RT-PCR and fragment analysis. RESULTS In this study, a patient with a long history of tobacco use was reported with a diagnosis of simultaneous low-grade B-cell lymphoproliferative disorder (LPD) and myeloproliferative neoplasm (MPN). Mutational analysis revealed that JAK2 V617F mutation and CALR mutation with 52bp deletion were present in this patient. CONCLUSION These results suggest that lymphoproliferative and myeloproliferative neoplasms may coexist, although the pathogenetic mechanism of coexisting hematologic requires further investigation. Additionally, the data indicate that JAK2 V617F and CALR mutations are not
https://www.semanticscholar.org/paper/Coexistence-of-lymphoproliferative-and-myeloprolif-Yang/280a8e2fa3c39d4514027f9e3b73240270e5b61e
Myeloproliferative neoplasm with ETV6-ABL1 fusion: a case report and literature review | Molecular Cytogenetics | Full TextMyeloproliferative neoplasm with ETV6-ABL1 fusion: a case report and literature review | Molecular Cytogenetics | Full Text
ETV6-ABL1 is a rare gene fusion with oncogenic properties, reported so far in 28 patients presenting a variety of haematological malignancies associated with clinical outcome, including chronic myeloid leukaemia (CML), acute myeloid leukaemia (AML), acute lymphoblastic leukaemia (ALL) and chronic myeloproliferative neoplasm (cMPN). Here we report on a 46-year-old female who presented with Philadelphia negative CML, positive for the ETV6-ABL1 fusion. Whole genome screening carried out with oligonucleotide arrays showed a subtle loss at 12p13 and cryptic imbalances within the 9q34.3 region in a highly unstable genome. FISH mapping with custom BAC probes identified two breakpoints 5 Mb apart within the 9q34 region, together with a break at 12p13. While FISH with commercial BCR-ABL1 probes failed to detect any ABL1 changes, the ETV6 break-apart probe conclusively identified the ETV6-ABL1 fusion thus determining the probes role as the primary diagnostic FISH test for this chimeric oncogene. In addition, we
https://molecularcytogenetics.biomedcentral.com/articles/10.1186/1755-8166-6-39
Myeloproliferative Neoplasm (MPN) Symptom Assessment Form Total Symptom Score : Prospective International Assessment of an...Myeloproliferative Neoplasm (MPN) Symptom Assessment Form Total Symptom Score : Prospective International Assessment of an...
PURPOSE Myeloproliferative neoplasm (MPN) symptoms are troublesome to patients, and alleviation of this burden represents a paramount treatment objective in the development of MPN-directed therapies. We aimed to assess the utility of an abbreviated symptom score for the most pertinent and representative MPN symptoms for subsequent serial use in assessing response to therapy.. PATIENTS AND METHODS. The Myeloproliferative Neoplasm Symptom Assessment Form total symptom score (MPN-SAF TSS) was calculated as the mean score for 10 items from two previously validated scoring systems. Questions focus on fatigue, concentration, early satiety, inactivity, night sweats, itching, bone pain, abdominal discomfort, weight loss, and fevers.. RESULTS MPN-SAF TSS was calculable for 1,408 of 1,433 patients with MPNs who had a mean score of 21.2 (standard deviation [SD], 16.3). MPN-SAF TSS results significantly differed among MPN disease subtypes (P , .001), with a mean of 18.7 (SD, 15.3), 21.8 (SD, 16.3), and 25.3 ...
http://uu.diva-portal.org/smash/record.jsf?pid=diva2:572120
Molecular Pathogenesis of Philadelphia-Positive Chronic Myeloid Leukemia - is it all BCR-ABL? | Bentham ScienceMolecular Pathogenesis of Philadelphia-Positive Chronic Myeloid Leukemia - is it all BCR-ABL? | Bentham Science
Title: Molecular Pathogenesis of Philadelphia-Positive Chronic Myeloid Leukemia - is it all BCR-ABL?. VOLUME: 11 ISSUE: 1. Author(s):H. Rumpold and G. Webersinke. Affiliation:Department of Haematology and Medical Oncology, Hospital Barmherzige Schwestern Linz, Seilerstaette 4, 4010 Linz, Austria.. Keywords:BCR-ABL, CML, pathogenesis, molecular genetics, chronic myeloproliferative diseases, Chronic Myelogenous Leukemia, Leukemia, Mastocytosis, diagnostic, chromosome, oncogen, stem cells, MOLECULAR BIOLOGY, cytoplasm, tyrosine kinase, plekstrine homology domain, toxin substrate, phosphorylation of tyrosine, immune system, bone marrow cells, haematopoiesis, pulmonary haemorrhages, PH-TRANSLOCATION, risk factor, phenotype, heterozygous, myeloproliferative nepolasms, myeloid colony, phenylalanine, leukemic cell, cell, phosphorylation, antiapoptotic protein, michochondrial cytochrome-c, mRNA, disease progression, Granulocytemacrophage progenitor cells, mutation rate, clonogenicity potential, STEM ...
https://www.eurekaselect.com/72940/article
Effect of mutation order on myeloproliferative neoplasms. - Oxford Big Data Institute
BACKGROUND: Cancers result from the accumulation of somatic mutations, and their properties are thought to reflect the sum of these mutations. However, little is known about the effect of the order in which mutations are acquired. METHODS: We determined mutation order in patients with myeloproliferative neoplasms by genotyping hematopoietic colonies or by means of next-generation sequencing. Stem cells and progenitor cells were isolated to study the effect of mutation order on mature and immature hematopoietic cells. RESULTS: The age at which a patient presented with a myeloproliferative neoplasm, acquisition of JAK2 V617F homozygosity, and the balance of immature progenitors were all influenced by mutation order. As compared with patients in whom the TET2 mutation was acquired first (hereafter referred to as TET2-first patients), patients in whom the Janus kinase 2 (JAK2) mutation was acquired first (JAK2-first patients) had a greater likelihood of presenting with polycythemia vera than with
https://www.bdi.ox.ac.uk/publications/623509
MYELOPROLIFERATIVE DISORDERS OR POORLY DIFFERENTIATED NEOPLASMS WITH MAJOR O.R. PROCEDURE WITHOUT CC/MCC - DRG Code 828MYELOPROLIFERATIVE DISORDERS OR POORLY DIFFERENTIATED NEOPLASMS WITH MAJOR O.R. PROCEDURE WITHOUT CC/MCC - DRG Code 828
The DRG code for MYELOPROLIFERATIVE DISORDERS OR POORLY DIFFERENTIATED NEOPLASMS WITH MAJOR O.R. PROCEDURE WITHOUT CC/MCC is 828. DRG code 828 is classified under DRG code range Myeloproliferative Diseases & Disorders, Poorly Differentiated Neoplasms.
https://coder.aapc.com/drg-codes/828
Myeloproliferative Neoplasm Causes, Types, Treatment, Symptoms & SignsMyeloproliferative Neoplasm Causes, Types, Treatment, Symptoms & Signs
Myeloproliferative Neoplasm Causes, Types, Treatment, Symptoms & Signs. Click for basic information about myeloproliferative neoplasm, its symptoms and treatment.
https://massivebio.com/myeloproliferative-neoplasm-basics
Myeloproliferative Neoplasms Treatment Market Gross Margin, Attractiveness, Competitive Landscape and Key Players - Crypto...
Coherent Market Insights recently published a detailed study of Myeloproliferative Neoplasms Treatment Market covering interesting aspects of the market with supporting development scenarios ranging from 2018-2026. The report delivers the clean elaborated structure of the Market comprising each and every business-related information of the market at a global level. The complete range of information related to the Global Market is obtained through various sources and this obtained bulk of the information is arranged, processed, and represented by a group of specialists through the application of different methodological techniques and analytical tools such as SWOT analysis to generate a whole set of trade-based study regarding the Myeloproliferative Neoplasms Treatment.. Get Free Download PDF Brochure: https://www.coherentmarketinsights.com/insight/request-pdf/930. This report assesses the growth rate and the market value on the basis of the key market dynamics, as well as the growth inducing ...
https://cryptonewsgazette.com/myeloproliferative-neoplasms-treatment-market-gross-margin-attractiveness-competitive-landscape-and-key-players/
Cloning and characterization of a novel druggable fusion kinase in acute myeloid leukemia | HaematologicaCloning and characterization of a novel druggable fusion kinase in acute myeloid leukemia | Haematologica
Rearrangements of the genes for the platelet-derived growth factor receptor alpha (PDGFRa) or the platelet-derived growth factor receptor beta (PDGFRβ) have been identified in myeloid/lymphoid neoplasms with eosinophilia.1 Fusions of PDGFRβ, which encodes a type III receptor tyrosine kinase, result in constitutive kinase activation and promotion of survival, proliferation and cell migration.2 Since the description of ETV6-PDGFRβ in 1994,3 more than 30 PDGFRβ fusion partners have been described, most of which have been identified in myeloproliferative neoplasms or in a myelodysplastic syndrome/myeloproliferative neoplasm overlap, but only rarely in acute myeloid leukemia (AML).541. PDGFRβ fusions are commonly in-frame mutations that contain an N-terminal fusion partner with dimerization/oligomerization motifs, enabling PDGFR-ligand-independent receptor tyrosine kinase activation.61 PDGFRβ-rearranged chronic myeloproliferative neoplasms show a favorable response to imatinib with a 10- year ...
https://haematologica.org/article/view/9993
Improved survival for patients with chronic blood diseases | News | News | Karolinska InstitutetImproved survival for patients with chronic blood diseases | News | News | Karolinska Institutet
New research from Karolinska Institutet shows that the survival for patients with chronic myeloproliferative diseases has improved in recent decades. This is despite the fact that no targeted drugs have yet been registered for this group of diseases. More than 9,000 patients have been included in a unique population-based study which has been published in the Journal of Clinical Oncology.
https://ki.se/en/news/improved-survival-for-patients-with-chronic-blood-diseases
Plus itPlus it
The recent discovery of an acquired activating point mutation in JAK2, substituting valine at amino acid position 617 for phenylalanine, has greatly improved our understanding of the molecular mechanism underlying chronic myeloproliferative neoplasms. Strikingly, the JAK2V617F mutation is found in nearly all patients suffering from polycythemia vera and in roughly every second patient suffering from essential thrombocythemia and primary myelofibrosis. Thus, JAK2 represents a promising target for the treatment of myeloproliferative neoplasms and considerable efforts are ongoing to discover and develop inhibitors of the kinase. Here, we report potent inhibition of JAK2V617F and JAK2 wild-type enzymes by a novel substituted quinoxaline, NVP-BSK805, which acts in an ATP-competitive manner. Within the JAK family, NVP-BSK805 displays more than 20-fold selectivity towards JAK2 in vitro, as well as excellent selectivity in broader kinase profiling. The compound blunts constitutive STAT5 phosphorylation ...
http://mct.aacrjournals.org/content/early/2010/06/24/1535-7163.MCT-10-0053
Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®): Treatment - Patient Information [NCI] - WellSpan Health LibraryMyelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®): Treatment - Patient Information [NCI] - WellSpan Health Library
Myelodysplastic/myeloproliferative neoplasms are a group of diseases in which the bone marrow makes too many white blood cells. Myelodysplastic /myeloproliferative neoplasms are diseases of the blood and bone marrow. Anatomy of the bone. The bone is made up of compact bone, spongy bone, and bone marrow. Compact bone...
http://www.wellspan.org/health-library/Document.aspx?id=ncicdr0000378291
Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®): Treatment - Patient Information [NCI] - North Kansas City...Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®): Treatment - Patient Information [NCI] - North Kansas City...
Myelodysplastic/myeloproliferative neoplasms are a group of diseases in which the bone marrow makes too many white blood cells. Myelodysplastic /myeloproliferative neoplasms are diseases of the blood and bone marrow. Anatomy of the bone. The bone is made up of compact bone, spongy bone, and bone marrow. Compact bone...
https://www.nkch.org/patients-visitors/health-library/healthwise-document-viewer/?id=ncicdr0000378291
Somatic mutations of calreticulin in myeloproliferative neoplasms and myelodysplastic/myeloproliferative neoplasms<...
TY - JOUR. T1 - Somatic mutations of calreticulin in myeloproliferative neoplasms and myelodysplastic/myeloproliferative neoplasms. AU - Malcovati, Luca. AU - Rumi, Elisa. AU - Cazzola, Mario. PY - 2014/11/1. Y1 - 2014/11/1. UR - http://www.scopus.com/inward/record.url?scp=84908457975&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84908457975&partnerID=8YFLogxK. U2 - 10.3324/haematol.2014.113944. DO - 10.3324/haematol.2014.113944. M3 - Article. C2 - 25420280. AN - SCOPUS:84908457975. VL - 99. SP - 1650. EP - 1652. JO - Haematologica. JF - Haematologica. SN - 0390-6078. IS - 11. ER - ...
https://moh-it.pure.elsevier.com/en/publications/somatic-mutations-of-calreticulin-in-myeloproliferative-neoplasms-2
8p11 Myeloproliferative Syndrome disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials8p11 Myeloproliferative Syndrome disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials
Genetics Home Reference : 25 8p11 myeloproliferative syndrome is a blood cancer that involves different types of blood cells. Blood cells are divided into several groups (lineages) based on the type of early cell from which they are descended. Two of these lineages are myeloid cells and lymphoid cells. Individuals with 8p11 myeloproliferative syndrome can develop both myeloid cell cancer and lymphoid cell cancer ...
http://www.malacards.org/card/8p11_myeloproliferative_syndrome
Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. - Centre for Tropical Medicine and Global...
Human myeloproliferative disorders form a range of clonal haematological malignant diseases, the main members of which are polycythaemia vera, essential thrombocythaemia, and idiopathic myelofibrosis. The molecular pathogenesis of these disorders is unknown, but tyrosine kinases have been implicated in several related disorders. We investigated the role of the cytoplasmic tyrosine kinase JAK2 in patients with a myeloproliferative disorder.We obtained DNA samples from patients with polycythaemia vera, essential thrombocythaemia, or idiopathic myelofibrosis. The coding exons of JAK2 were bidirectionally sequenced from peripheral-blood granulocytes, T cells, or both. Allele-specific PCR, molecular cytogenetic studies, microsatellite PCR, Affymetrix single nucleotide polymorphism array analyses, and colony assays were undertaken on subgroups of patients.A single point mutation (Val617Phe) was identified in JAK2 in 71 (97%) of 73 patients with polycythaemia vera, 29 (57%) of 51 with essential
https://www.tropicalmedicine.ox.ac.uk/publications/998683
Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. - Centre for Tropical Medicine and Global...
Human myeloproliferative disorders form a range of clonal haematological malignant diseases, the main members of which are polycythaemia vera, essential thrombocythaemia, and idiopathic myelofibrosis. The molecular pathogenesis of these disorders is unknown, but tyrosine kinases have been implicated in several related disorders. We investigated the role of the cytoplasmic tyrosine kinase JAK2 in patients with a myeloproliferative disorder.We obtained DNA samples from patients with polycythaemia vera, essential thrombocythaemia, or idiopathic myelofibrosis. The coding exons of JAK2 were bidirectionally sequenced from peripheral-blood granulocytes, T cells, or both. Allele-specific PCR, molecular cytogenetic studies, microsatellite PCR, Affymetrix single nucleotide polymorphism array analyses, and colony assays were undertaken on subgroups of patients.A single point mutation (Val617Phe) was identified in JAK2 in 71 (97%) of 73 patients with polycythaemia vera, 29 (57%) of 51 with essential
https://www.tropicalmedicine.ox.ac.uk/publications/998683/
DMOZ - Health: Conditions and Diseases: Cancer: Hematologic: Chronic Myeloproliferative Disorders
Disorders which share common features: Polycythemia Vera causes the overproduction of red cells, white cells and platelets; Idiopathic Myelofibrosis has an overproduction of white cells or platelets with anemia and an increase in bone marrow fibrous tissue. Essential Thrombocytosis features an increase in circulating platelets. Also Chronic Neutrophilic Leukemia and Chronic Eosinophilic Leukemia both which may develop into Leukemia.
http://dmoztools.net/Health/Conditions_and_Diseases/Cancer/Hematologic/Chronic_Myeloproliferative_Disorders/
Myelophthisic anemia - WikipediaMyelophthisic anemia - Wikipedia
Myelophthisic anemia (or myelophthisis) is a severe type of anemia found in some people with diseases that affect the bone marrow. Myelophthisis refers to the displacement of hemopoietic bone-marrow tissue either by fibrosis, tumors or granulomas. The word comes from the roots myelo-, which refers to bone marrow, and phthysis, shrinkage or atrophy. Myelophthisis can occur in the setting of chronic myeloproliferative disease (e.g. myelofibrosis), leukemia, lymphoma, and metastatic carcinoma or myeloma. It is common in people who have chronic idiopathic myelofibrosis. It has been linked to small-cell lung cancer, breast cancer or prostate cancer that metastasizes to the bone marrow. Historically, the most common cause of displacement of healthy bone marrow was tuberculosis.[citation needed] Currently, the most common cause is displacement of bone marrow by metastatic cancer (extramedullary hematopoiesis tends to be modest). Other causes include myeloproliferative disorders (especially late-stage ...
https://en.wikipedia.org/wiki/Myelophthisic_anemia
MPL W515L/K Mutations in Chronic Myeloproliferative Neoplasms [Turk J Hematol]MPL W515L/K Mutations in Chronic Myeloproliferative Neoplasms [Turk J Hematol]
RESULTS: In our study, MPL W515L/K or JAK-2 V617F mutations were not observed in healthy controls. JAK-2 V617F mutation was present in 35 patients, of whom 29 had ET (43.9%, 29/66) and 6 had PMF (54.5%, 6/11). In the patient group, MPL W515L/K mutations were found in only 2 PMF cases, and these cases were negative for JAK-2 V617F mutation. The prevalence of MPL W515L/K mutations in the patient group was 2.6%, and the prevalence of MPL W515L/K mutations among the cases negative for the JAK-2 V617F mutation was found to be 4.8%. The 2 cases with MPL W515L/K mutations had long follow-up times (124 months and 71 months, respectively), had no thrombotic or hemorrhagic complications, and had no additional cytogenetic anomalies ...
http://www.tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-65807&look4=
Polycythemia vera | Multimedia Encyclopedia | Health Information | St. Lukes HospitalPolycythemia vera | Multimedia Encyclopedia | Health Information | St. Lukes Hospital
Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. The polycythemias. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice . 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 67. National Cancer Institute: PDQ Chronic Myeloproliferative Neoplasms Treatment: Polycythemia Vera. Bethesda, MD: National Cancer Institute. Date last modified December 3, 2014. Available at: www.cancer.gov/cancertopics/pdq/treatment/myeloproliferative/HealthProfessional/page3. Accessed March 3, 2015.. Tefferi A. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Schafer AI, eds. Goldmans Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 166. ...
https://www.stlukes-stl.com/health-content/health-ency-multimedia/1/000589.htm
Characterization and Prognosis Significance of JAK2 (V617F), MPL, and CALR Mutations in Philadelphia-Negative...Characterization and Prognosis Significance of JAK2 (V617F), MPL, and CALR Mutations in Philadelphia-Negative...
Background: The discovery of somatic acquired mutations of JAK2 (V617F) in Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) has not only improved rational disease classification and prognostication but also brings new understanding insight into the pathogenesis of diseases. Dosage effects of the JAK2 (V617F) allelic burden in Ph-negative MPNs may partially influence clinical presentation, disease progression, and treatment outcome. Material and Methods: Pyrosequencing was performed to detect JAK2 (V617F) and MPL (W515K/L) and capillary electrophoresis to identify CALR exon 9.0 mutations in 100.0 samples of Ph-negative MPNs (38.0 PV, 55 ET, 4 PMF, and 3 MPN-U). Results: The results showed somatic mutations of JAK2 (V617F) in 94.7% of PV, 74.5% of ET, 25.0% of PMF, and all MPN-U. A high proportion of JAK2 (V617F) mutant allele burden (mutational load | 50.0%) was predominantly observed
http://journal.waocp.org/article_40503.html
Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable - DrugBankMyelodysplastic/Myeloproliferative Neoplasm, Unclassifiable - DrugBank
This project is supported by the Canadian Institutes of Health Research (award #111062), Alberta Innovates - Health Solutions, and by The Metabolomics Innovation Centre (TMIC), a nationally-funded research and core facility that supports a wide range of cutting-edge metabolomic studies. TMIC is funded by Genome Alberta, Genome British Columbia, and Genome Canada, a not-for-profit organization that is leading Canadas national genomics strategy with funding from the federal government. Maintenance, support, and commercial licensing is provided by OMx Personal Health Analytics, Inc. Designed by Educe Design & Innovation Inc. ...
https://www.drugbank.ca/indications/DBCOND0029236
Polycythemia veraPolycythemia vera
Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. The polycythemias. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 68. National Cancer Institute website. Chronic myeloproliferative neoplasms treatment (PDQ) -- health professional version. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/_5. Updated February 1, 2019. Accessed March 1, 2019. Tefferi A. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 166. BACK TO TOP Review Date: 1/29/2019 Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.. ...
http://slu.adam.com/content.aspx?productid=617&pid=1&gid=000589
Jerry Spivak LabJerry Spivak Lab
Research in the Jerry Spivak Lab focuses on chronic myeloproliferative disorders, particularly their molecular mechanisms and methods for distinguishing them diagnostically and interventionally. By analyzing gene expression in polycythemia vera stem cells, we have learned that patients with polycythemia vera can be differentiated from those with erythrocytosis and can be diagnosed as having either aggressive or slow-growing disease. We are also studying the roles played by specific molecular markers in the pathogenesis and diagnosis of polycythemia vera.. Research Areas: stem cells, pathogenesis, polycythemia vera, myeloproliferative disorders ...
https://www.hopkinsmedicine.org/research/labs/jerry-spivak-lab
MPL mutations in myeloproliferative disorders: analysis of the PT-1 cohort. - Clinical Trial Service Unit & Epidemiological...
Activating mutations of MPL exon 10 have been described in a minority of patients with idiopathic myelofibrosis (IMF) or essential thrombocythemia (ET), but their prevalence and clinical significance are unclear. Here we demonstrate that MPL mutations outside exon 10 are uncommon in platelet cDNA and identify 4 different exon 10 mutations in granulocyte DNA from a retrospective cohort of 200 patients with ET or IMF. Allele-specific polymerase chain reaction was then used to genotype 776 samples from patients with ET entered into the PT-1 studies. MPL mutations were identified in 8.5% of JAK2 V617F(-) patients and a single V617F(+) patient. Patients carrying the W515K allele had a significantly higher allele burden than did those with the W515L allele, suggesting a functional difference between the 2 variants. Compared with V617F(+) ET patients, those with MPL mutations displayed lower hemoglobin and higher platelet levels at diagnosis, higher serum erythropoietin levels, endogenous megakaryocytic but
https://www.ctsu.ox.ac.uk/publications/327255
Basophil production in myeloproliferative disorders: increases during acute blastic transformation of chronic myeloid leukemia ...Basophil production in myeloproliferative disorders: increases during acute blastic transformation of chronic myeloid leukemia ...
JA Denburg, WE Wilson, J Bienenstock; Basophil production in myeloproliferative disorders: increases during acute blastic transformation of chronic myeloid leuk
https://ashpublications.org/blood/article/60/1/113/163262/Basophil-production-in-myeloproliferative
Idiopathic myelofibrosis: dental treatment considerations.<...Idiopathic myelofibrosis: dental treatment considerations.<...
TY - JOUR. T1 - Idiopathic myelofibrosis. T2 - dental treatment considerations.. AU - Steelman, Robert. AU - Holmes, D.. AU - Cranston, R.. AU - Cupp, D.. PY - 1991/3. Y1 - 1991/3. N2 - Idiopathic myelofibrosis is a myeloproliferative disorder of unknown origin. The bone marrow becomes fibrotic with an associated decrease in hematopoiesis resulting in anemia, bleeding problems, splenomegaly, and other secondary abnormalities. Although idiopathic myelofibrosis is usually diagnosed in middle age, there have been a few reports of the disorder in the pediatric population. This case report documents dental treatment considerations in a 6-year-old female with idiopathic myelofibrosis, severe anemia, and abnormal blood coagulation studies. The patient was successfully treated in a hospital after medical consultation, transfusion of packed red blood cells, and administration of prophylactic antibiotics. Local hemostatic measures following multiple extractions of carious teeth controlled bleeding. No ...
https://ohsu.pure.elsevier.com/en/publications/idiopathic-myelofibrosis-dental-treatment-considerations-2
Most recent papers with the keyword Driver mutation | Read by QxMDMost recent papers with the keyword Driver mutation | Read by QxMD
BACKGROUND: The classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), consisting of polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are a heterogeneous group of neoplasms that harbor driver mutations in the JAK2, CALR, and MPL genes. The detection of these mutations has been incorporated into the recent World Health Organization (WHO) diagnostic criteria for MPN. Given a pressing clinical need to screen for these mutations in a routine diagnostic setting, a targeted next-generation sequencing (NGS) assay for the detection of MPN-associated mutations located in JAK2 exon 14, JAK2 exon 12, CALR exon 9, and MPL exon 10 was developed to provide a single platform alternative to reflexive, stepwise diagnostic algorithms ...
https://www.readbyqxmd.com/keyword/110603
Search Results | JNCCNSearch Results | JNCCN
The classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPN), which include essential thrombocythemia, polycythemia vera, and myelofibrosis (MF), are in a new era of molecular diagnosis, ushered in by the identification of the JAK2 V617F and cMPL mutations in 2005 and 2006, respectively, and the CALR mutations in 2013. Coupled with increased knowledge of disease pathogenesis and refined diagnostic criteria and prognostic scoring systems, a more nuanced appreciation has emerged of the burden of MPN in the United States, including the prevalence, symptom burden, and impact on quality of life. Biological advances in MPN have translated into the rapid development of novel therapeutics, culminating in the approval of the first treatment for MF, the JAK1/JAK2 inhibitor ruxolitinib. However, certain practical aspects of care, such as those regarding diagnosis, prevention of vascular events, choice of cytoreductive agent, and planning for therapies, present challenges for ...
https://jnccn.org/search?f_0=author&q_0=Bart+Scott&print
EHA-SWG Scientific Meeting on Challenges in the Diagnosis and Management of Myeloproliferative NeoplasmsEHA-SWG Scientific Meeting on Challenges in the Diagnosis and Management of Myeloproliferative Neoplasms
Dates: October 12-14, 2017Location: Budapest, HungaryChairs: C Harrison, JJ Kiladjian EHA and the EHA Scientific Working Group on Myeloproliferative Neoplasms are organizing this EHA-SWG Scientific Meeting on Challenges in the Diagnosis and Management of ...
https://ehaweb.org/meetings/swg-mpn/
Duas classes de mutação na evolução de policitemia vera para leucemia mielóide aguda
Polycythemia vera (PV) is a chronic myeloproliferative disorder that can evolve to marrow fibrosis or acute leukemia (AML). Cytogenetic alterations can be detected in around 25% of patients at diagnosis and in up to 50% of those with progression. We report a case of PV with evolution to AML in which it was possible to demonstrate the two-hit model of leukemogenesis: one mutation confers proliferative advantage and another interferes with differentiation. Case: A 55-year-old female patient was diagnosed with PV in 2002 and treated with phlebotomies and hydroxyurea. In 2006, there was progression topost-polycythemic fibrosis with AML one year later. She presented the JAK2V617F mutation. The result of karyotyping performed at diagnosis was normal and at transformation, 46,XX,del(20)(q13.1) was detected in 4/20 metaphases. FISH analysis of a stored sample for 20q13 showed the deletion in 20% of interphases confirming the earlier presence of a clonal abnormality that was not detected by karyotyping. ...
http://repositorio.unifesp.br/handle/11600/4991
Updates for the Advanced Practitioner - Myeloproliferative Neoplasms
Collectively, the studies discussed demonstrate that occasional patients with CALR mutation-positive essential thrombocythemia or myelofibrosis carry other myeloproliferative neoplasms-initiating genetic mutations (including JAK2 V617F), acquire secondary mutations before or after the CALR mutation, or evolve over time to being CALR mutation-homozygous.. Br J Haematol ...
https://updates.advancedpractitioner.com/myeloproliferative-neoplasms/news-and-literature-highlights/progenitor-genotyping-reveals-a-complex-clonal-architecture/
JCI - Conditional expression of oncogenic K-ras from its endogenous promoter induces a myeloproliferative diseaseJCI - Conditional expression of oncogenic K-ras from its endogenous promoter induces a myeloproliferative disease
Oncogenic ras alleles are among the most common mutations found in patients with acute myeloid leukemia (AML). Previously, the role of oncogenic ras in cancer was assessed in model systems overexpressing oncogenic ras from heterologous promoters. However, there is increasing evidence that subtle differences in gene dosage and regulation of gene expression from endogenous promoters play critical roles in cancer pathogenesis. We characterized the role of oncogenic K-ras expressed from its endogenous promoter in the hematopoietic system using a conditional allele and IFN-inducible, Cre-mediated recombination. Mice developed a completely penetrant myeloproliferative syndrome characterized by leukocytosis with normal maturation of myeloid lineage cells; myeloid hyperplasia in bone marrow; and extramedullary hematopoiesis in the spleen and liver. Flow cytometry confirmed the myeloproliferative phenotype. Genotypic and Western blot analysis demonstrated Cre-mediated excision and expression, ...
https://taoyantao.com.www.mobile.jci.org/articles/view/20476/pdf
Example of an examinationExample of an examination
30 REPRESENTATIVE TEST QUESTIONS FROM PREVIOUS EXAMINATIONS Including answers, in each question only one answer is correct 1 Which of the following bone marrow diseases is considered a chronic myeloproliferative disorder a b c d e Chronic myelomonocytic leukaemia Essential thrombocythaemia Hypocellular myelodysplastic syndrome Juvenile chronic myeloid leukaemia Refractory anaemia with excess of blasts in…
https://europathol.wordpress.com/board-exam/example-of-an-examination/
Myeloproliferative Disorders and MyelofibrosisMyeloproliferative Disorders and Myelofibrosis
Ruxolitinib (Jakafi), an oral JAK1 and JAK2 kinase inhibitor, was approved in November 2011 for the treatment of patients with intermediate or high-risk myelofibrosis, including primary myelofibrosis, post-polycythemia vera myelofibrosis, and post-essential thrombocythemia myelofibrosis.14 Two phase III trials demonstrated significant improvement compared with best available therapy for spleen size, symptoms, and burden reduction, as well as for quality of life.14-16 However, the reduced spleen size was not shown to be consistently durable in a phase I/II study and neither phase III study reported a significant survival benefit.17,18 This agent is commonly associated with hematologic side effects; anemia was reported in 96% of patients taking ruxolitinib (grade 3/4, 45%), and thrombocytopenia was reported in 70% (grade 3/4, 13%).14 This first JAK inhibitor therapy for myelofibrosis has been long anticipated; yet, the value of this treatment is not truly known. The treatment of adverse events and ...
https://www.ajmc.com/journals/evidence-based-oncology/2012/2012-2-vol18-n3/myeloproliferative-disorders-and-myelofibrosis
Update on the randomized trial of post-transplanation cyclophosphamide and rabbit ATG for graft-versus-host disease prophylaxis...Update on the randomized trial of post-transplanation cyclophosphamide and rabbit ATG for graft-versus-host disease prophylaxis...
Промежуточные результаты рандомизированного исследования посттрансплантационного циклофос- фана и кроличьего АТГ в качестве профилактики реакции трансплантат против хозяина у пациентов с миелопролиферативными заболеваниями и мие- лодиспластическим синдромUpdate on the randomized trial of post-transplanation cyclophosphamide and rabbit ATG for graft-versus-host disease prophylaxis in chronic myeloproliferative neoplasms and myelodysplastic syndrome
http://cttjournal.com/en/archive/vypusk-5-nomer-3/korotkie-soobshcheniya/promezhutochnye-rezultaty-randomizirovannogo-issledovaniya-posttransplantatsionnogo-tsiklofos-fana-i/
Mutagenetix > Incidental...Mutagenetix > Incidental...
FUNCTION: [Summary is not available for the mouse gene. This summary is for the human ortholog.] This gene encodes a cell surface tyrosine kinase receptor for members of the platelet-derived growth factor family. These growth factors are mitogens for cells of mesenchymal origin. The identity of the growth factor bound to a receptor monomer determines whether the functional receptor is a homodimer or a heterodimer, composed of both platelet-derived growth factor receptor alpha and beta polypeptides. This gene is flanked on chromosome 5 by the genes for granulocyte-macrophage colony-stimulating factor and macrophage-colony stimulating factor receptor; all three genes may be implicated in the 5-q syndrome. A translocation between chromosomes 5 and 12, that fuses this gene to that of the translocation, ETV6, leukemia gene, results in chronic myeloproliferative disorder with eosinophilia. [provided by RefSeq, Jul 2008 ...
https://mutagenetix.utsouthwestern.edu/incidental/incidental_rec.cfm?mid=156418
Mutagenetix > Incidental...Mutagenetix > Incidental...
FUNCTION: [Summary is not available for the mouse gene. This summary is for the human ortholog.] This gene encodes a cell surface tyrosine kinase receptor for members of the platelet-derived growth factor family. These growth factors are mitogens for cells of mesenchymal origin. The identity of the growth factor bound to a receptor monomer determines whether the functional receptor is a homodimer or a heterodimer, composed of both platelet-derived growth factor receptor alpha and beta polypeptides. This gene is flanked on chromosome 5 by the genes for granulocyte-macrophage colony-stimulating factor and macrophage-colony stimulating factor receptor; all three genes may be implicated in the 5-q syndrome. A translocation between chromosomes 5 and 12, that fuses this gene to that of the translocation, ETV6, leukemia gene, results in chronic myeloproliferative disorder with eosinophilia. [provided by RefSeq, Jul 2008 ...
https://mutagenetix.utsouthwestern.edu/incidental/incidental_rec.cfm?mid=274333
美国APExBIO中文官网 - ITF2357 (Givinostat)|HDAC inhibitor|CAS# 732302-99-7美国APExBIO中文官网 - ITF2357 (Givinostat)|HDAC inhibitor|CAS# 732302-99-7
ITF2357, also known as givinostat, is a potent inhibitor of both class I and class II histone deacetylase (HDAC) as well as a potent inhibitor of hematopoietic colony formation by JAKEV617F-bearing progenitor cells from chronic myeloproliferative neoplasm
http://www.apexbio.cn/itf2357-givinostat.html
Microsoft word - idrossiurea eha 2011.doc
SIDE EFFECTS OF HYDROXYUREA IN CLASSIC CHRONIC MYELOPROLIFERATIVE NEOPLASMS. A RETROSPECTIVE STUDY OF 3,411 PATIENTS E Antonioli 1, P Guglielmelli 2, L Pieri 2, MC Finazzi 3, E Rumi 4, V Martinelli 5, N Vianelli 6, ML Randi 7, I Bertozzi7 , V De Stefano 8, T Za 8,M Ruggeri 9, F Rodeghiero 9, E Elli 10, E Pogliani 10, R Cacciola 11, E Cacciola 11, G Leone 8, M Baccarani6 , F Passamonti 4, G Finazzi 3, A Rambaldi 3, A Bosi2, T Barbui 3, A Vannucchi 2 1- AOU Careggi, Firenze, Italy ...
http://pdfmedsearch.com/p/progettoagimm.it1.html
Gifts: 1/17/09Gifts: 1/17/09
When I was around 5 months pregnant an ultrasound revealed a birth defect (duodenal atresia - a blockage between the intestines and stomach) in Tomas which also meant he had a high chance of being a Down Syndrome baby. About a month before he was born I had an amnio that showed he did indeed have DS. He was born on January 16, 2009 and had his first surgery when he was 32 hours old. After that, test result after test result rolled in. In the first month my family learned he had three holes in his heart, his liver was not working, and he had Transient Myeloproliferative disorder (a type of leukemia which resolves in the first few months of life). The second month revealed laryngomalacia (a collapsing larynx), primary and secondary aspiration, and severe reflux. He was switched to tube feedings and had his second surgery to correct the reflux that was causing him to suffocate. The TMD resolved when he was 4 months old, his liver started working when he was 5 months old, and the holes in his heart ...
http://mandd3.blogspot.com/2009/07/11709.html
Gifts: The (really) good and the (not too) badGifts: The (really) good and the (not too) bad
When I was around 5 months pregnant an ultrasound revealed a birth defect (duodenal atresia - a blockage between the intestines and stomach) in Tomas which also meant he had a high chance of being a Down Syndrome baby. About a month before he was born I had an amnio that showed he did indeed have DS. He was born on January 16, 2009 and had his first surgery when he was 32 hours old. After that, test result after test result rolled in. In the first month my family learned he had three holes in his heart, his liver was not working, and he had Transient Myeloproliferative disorder (a type of leukemia which resolves in the first few months of life). The second month revealed laryngomalacia (a collapsing larynx), primary and secondary aspiration, and severe reflux. He was switched to tube feedings and had his second surgery to correct the reflux that was causing him to suffocate. The TMD resolved when he was 4 months old, his liver started working when he was 5 months old, and the holes in his heart ...
http://mandd3.blogspot.com/2010/11/really-good-and-not-too-bad.html
Mutational Pattern Recognition in Chronic Myeloid Neoplasms (CMNs)Mutational Pattern Recognition in Chronic Myeloid Neoplasms (CMNs)
This presentation summarizes the findings of the Association for Molecular Pathology Chronic Myeloid Neoplasm Working Group. Chronic myeloid neoplasms (CMNs) are defined as a complex group of hematopoietic disorders, encompassing myelodysplastic syndromes (MDS), myeloproliferative neoplasms (MPNs), the overlap entities (MDS/MPNs), and systemic mastocytosis. The goal of this group was to distill the vast amounts of literature on the mutational profiles of the CMNs into high impact information that would aid molecular pathologists in the development of next generation sequencing (NGS) myeloid panels. This work identifies 34 genes as the minimum recommended testing list and provides information on the frequency of these genes in the various CMNs as well as their prognostic and therapeutic import. In addition, the findings highlight the recurrent patterns of mutational clonal evolution in these patients, uncovering critical insight into the biology of these neoplasms.. Learning Objectives:. ...
https://educate.amp.org/local/catalog/view/product.php?productid=131
Polycythemia vera ultrasound - wikidocPolycythemia vera ultrasound - wikidoc
Abdominal ultrasound may be helpful in the diagnosis of myeloproliferative neoplasm. Findings on abdominal ultrasound suggestive of myeloproliferative neoplasm include splenomegaly, abdominal fluid, and hepatic lesions. Ultrasound of the extremities can assist with diagnosis of deep vein thrombosis, which is commonly associated with high-risk polycythemia vera. ...
http://wikidoc.org/index.php/Polycythemia_vera_ultrasound
Difference between revisions of BME103:W930 Group5 l2 - OpenWetWareDifference between revisions of BME103:W930 Group5 l2 - OpenWetWare
Another form of leukemia, transient myeloproliferative leukemia, is identified with a heterozygous C to A transversion as well. In a 2002 leukemia journal written by Taketani et al., the RUNX1 gene was screened and studied in a sample group of 46 patients with down syndrome. These patients all had hematologic malignancies, meaning they were all affected by different cancers associated with bone marrow. Out of these patients, was identified with this C to A transversion and diagnosed with transient myeloproliferative leukemia 5 days after birth. However, the newborn patient died 12 months after birth. The newborn was never screened for acute myeloid leukemia. The conclusion here is that if there is an identified C-A mutation regarding the RUNX1 gene, then AML should be screened and tested for. An amniotic fluid test should be given to pregnant women in order to determine if their children carry the mutated gene associated with acute myeloid leukemia. http://omim.org/entry/151385#0008 ...
https://openwetware.org/wiki/?title=BME103:W930_Group5_l2&diff=661608&oldid=659344
Chronic Idiopathic Myelofibrosis Research Report - Forecast 2017 - 2025 | CultHub
New York, NY -- 01/12/2018 -- Idiopathic myelofibrosis is a chronic myelo-proliferative disorder and characterized by abnormal mutation of stem cells. This abnormal mutation of stem cells and excessive production of platelets result in development of fibrous tissues within the bone-marrow. This factor would ultimately negatively affect on the development of white blood cells (WBCs),…
https://culthub.com/entertainment/chronic-idiopathic-myelofibrosis-research-report-forecast-2017-2025/58937
9781107212589 - Diagnostic Techniques in Hematological Malignancies9781107212589 - Diagnostic Techniques in Hematological Malignancies
Contents: Preface; Part I. Diagnostic Techniques: 1. Morphology Wendy N. Erber; 2. Immunocytochemistry Wendy N. Erber; 3. Flow cytometry Maryalice Stetler-Stevenson and Constance M. Yuan; 4. Cytogenetics Christine J. Harrison and Claire Schwab; 5. Molecular genetics Ken Mills; Part II. Hematological Malignancies: 6. The integrated approach to the diagnosis of hematological malignancies Mike A. Scott and Wendy N. Erber; 7. Acute lymphoblastic leukemia Elaine Coustan-Smith and Dario Campana; 8. Acute myeloid leukemia David Grimwade; 9. Mature B cell leukemias Constantine S. Tam and Michael J. Keating; 10. Mature T cell and natural killer-cell leukemias Kaaren K. Reichard and Kathryn Foucar; 11. Lymphoma Jennifer Herrick and Ahmet Dogan; 12. Plasma cell neoplasms Rafael Fonseca and Riccardo Valdez; 13. Chronic myeloid leukemia Emma J. Gudgin and Brian Huntly; 14. Myeloproliferative neoplasms Philip A. Beer and Anthony R. Green; 15. Myelodysplastic syndromes and myelodysplastic/myeloproliferative ...
https://www.wisepress.com/ebooks/diagnostic-techniques-in-hematological-malignancies-9781107212589/
Overview of Polycythemia Vera: Choosing a Treatment Thats Right for You | Myeloproliferative Neoplasms | Patient PowerOverview of Polycythemia Vera: Choosing a Treatment Thats Right for You | Myeloproliferative Neoplasms | Patient Power
PV patients may show no signs for years, but treatment is essential to manage the disease. Tune in to hear Dr. David Snyder as he shares an overview of PV and available treatments.
https://www.patientpower.info/video/overview-of-polycythemia-vera-choosing-a-treatment-that-s-right-for-you
Primary Myelofibrosis: Practice Essentials, Pathophysiology, EtiologyPrimary Myelofibrosis: Practice Essentials, Pathophysiology, Etiology
Primary myelofibrosis is a clonal disorder arising from the neoplastic transformation of early hematopoietic stem cells. Older terms for this disorder include agnogenic myeloid metaplasia with myelofibrosis and chronic idiopathic myelofibrosis.
https://emedicine.medscape.com/article/197954-overview
Myeloproliferative Neoplasms and Myelofibrosis: New Treatment Guidelines - The ASCO PostMyeloproliferative Neoplasms and Myelofibrosis: New Treatment Guidelines - The ASCO Post
Ruben A. Mesa, MD, of the Mayo Clinic Cancer Center, discusses molecular abnormalities and their use in the diagnosis, risk stratification, and selection of treatment for myelofibrosis.. ...
https://www.ascopost.com/videos/2017-nccn-annual-conference/myeloproliferative-neoplasms-and-myelofibrosis-new-treatment-guidelines/
Updates for the Advanced Practitioner - Myeloproliferative Neoplasms
Focusing on patients with polycythemia vera who were hydroxyurea-resistant/intolerant patients and ineligible for clinical trials, a phase 3b, expanded treatment protocol study demonstrated that ruxolitinib is safe and effective for this patient population. The study reported ,50% spleen reduction was achieved in 86.7% of patients, and dose adjustment/interruption due to adverse events occurred in 37.9% and study drug discontinuation in 8.7% of patients.. Leukemia & Lymphoma ...
https://updates.advancedpractitioner.com/myeloproliferative-neoplasms/news-and-literature-highlights/safety-and-efficacy-findings-from-the-open-label-multicenter-phase-3b-expanded-treatment-protocol-study-of-ruxolitinib/
BasophiliaBasophilia
"Leukemia: Chronic Myeloproliferative Disorders". Pathology Thread. University of Virginia School of Medicine. Archived from the ... Basophilia, as it is primarily a secondary condition, is treated by addressing the causative disease or disorder. The ... If splenomegaly is detected, a myeloproliferative syndrome may be suspected. Intrinsically related symptoms such as fever, ... April 2017). "Proposed diagnostic criteria and classification of basophilic leukemias and related disorders". Leukemia. 31 (4 ...
https://en.wikipedia.org/wiki/Basophilia
GATA2 deficiencyGATA2 deficiency
Hasle H (December 2016). "Myelodysplastic and myeloproliferative disorders of childhood". Hematology. American Society of ... Emotional and behavioral disorders: Autism spectrum disorders, chronic headache. The GATA2 transcription factor contains two ... It is also the most common cause of hereditary bone marrow failure and may present with this disorder. GATA 2 deficiency has ... In 2011, however, all cases of both disorders were found to be caused by inactivating mutations in the GATA2 gene. Subsequently ...
https://en.wikipedia.org/wiki/GATA2_deficiency
White blood cell differentialWhite blood cell differential
Neutrophilia may also occur in myeloproliferative disorders. Neutropenia, meaning a low neutrophil count, may occur as a ... These cell types may be found in blood disorders and other pathological states. The manual differential can also identify ... ISBN 978-0-323-22545-8. Sa A. Wang; Robert P. Hasserjian (4 June 2018). Diagnosis of Blood and Bone Marrow Disorders. Springer ... can occur along with other white blood cell abnormalities in chronic myeloid leukemia and other myeloproliferative disorders. ...
https://en.wikipedia.org/wiki/White_blood_cell_differential
PlateletPlatelet
... one of the myeloproliferative neoplasms or certain other myeloid neoplasms. A disorder of platelet function is called a ... Congenital Disorders of adhesion Bernard-Soulier syndrome Disorders of activation Disorders of granule amount or release ... One can get a clue as to whether bleeding is due to a platelet disorder or a coagulation factor disorder by the characteristics ... "Acquired von Willebrand disease in myeloproliferative disorders". Leukemia & Lymphoma. 22 Suppl 1: 79-82. doi:10.3109/ ...
https://en.wikipedia.org/wiki/Platelet
CD177CD177
Kralovics R, Skoda RC (2005). "Molecular pathogenesis of Philadelphia chromosome negative myeloproliferative disorders". Blood ...
https://en.wikipedia.org/wiki/CD177
LARP4LARP4
Kralovics R, Skoda RC (January 2005). "Molecular pathogenesis of Philadelphia chromosome negative myeloproliferative disorders ...
https://en.wikipedia.org/wiki/LARP4
Chronic myelomonocytic leukemiaChronic myelomonocytic leukemia
Barbara J. Bain (2003). Chronic Myeloproliferative Disorders: Cytogenetic and Molecular Genetic Abnormalities. Karger ... and a myeloproliferative neoplasm (MPN); a disorder characterised by the overproduction of blood cells. For this reason, CMML ... Myeloproliferative CMML (>13x109 monocytes/L) has a reduced survival compared with myelodysplastic CMML. A platelet count of ... Hydroxyurea is a chemotherapy that is used in the myeloproliferative form of CMML to reduce cell numbers. Decitabine/ ...
https://en.wikipedia.org/wiki/Chronic_myelomonocytic_leukemia
Primary myelofibrosisPrimary myelofibrosis
It is one of the myeloproliferative disorders, diseases of the bone marrow in which excess cells are produced at some stage. ... Tefferi A (2003). "The forgotten myeloproliferative disorder: myeloid metaplasia". The Oncologist. 8 (3): 225-231. doi:10.1634/ ... Myelofibrosis can be a late complication of other myeloproliferative disorders, such as polycythemia vera, and less commonly, ... a user's guide for chronic myeloproliferative disorders". Expert Review of Anticancer Therapy. 11 (3): 403-414. doi:10.1586/era ...
https://en.wikipedia.org/wiki/Primary_myelofibrosis
Erythropoietin receptorErythropoietin receptor
Kralovics R, Skoda RC (Jan 2005). "Molecular pathogenesis of Philadelphia chromosome negative myeloproliferative disorders". ... sites for phosphatases that negatively affect EpoR signaling in order to prevent overactivation that may lead to such disorders ...
https://en.wikipedia.org/wiki/Erythropoietin_receptor
Von Hippel-Lindau tumor suppressorVon Hippel-Lindau tumor suppressor
Kralovics R, Skoda RC (January 2005). "Molecular pathogenesis of Philadelphia chromosome negative myeloproliferative disorders ... "Cardiopulmonary function in two human disorders of the hypoxia-inducible factor (HIF) pathway: von Hippel-Lindau disease and ...
https://en.wikipedia.org/wiki/Von_Hippel–Lindau_tumor_suppressor
Juvenile myelomonocytic leukemiaJuvenile myelomonocytic leukemia
Bastida P; García-Miñaúr S; Ezquieta B; Dapena J. L.; De Toledo Sanchez (2011). "Myeloproliferative disorder in Noonan syndrome ... The World Health Organization has included JMML in the category of myelodysplastic and myeloproliferative disorders. The ... may predispose to the development of JMML or a myeloproliferative disorder (MPD) associated with NS (MPD/NS) which resembles ... is a myelodysplastic and myeloproliferative disorder. The diagnostic criteria were originally laid down by Neimeyer et al. in ...
https://en.wikipedia.org/wiki/Juvenile_myelomonocytic_leukemia
CBL (gene)CBL (gene)
"Mutant Cbl proteins as oncogenic drivers in myeloproliferative disorders". Oncotarget. 2 (3): 245-50. doi:10.18632/oncotarget. ... Quips article describing CBL function at PDBe OMIM entries on NOONAN SYNDROME-LIKE DISORDER WITH OR WITHOUT JUVENILE ...
https://en.wikipedia.org/wiki/CBL_(gene)
MegakaryocyteMegakaryocyte
2005-04-28). "A gain-of-function mutation of JAK2 in myeloproliferative disorders". N Engl J Med. 352 (17): 1779-90. doi: ... The cause for this disorder appears to be a mutation in the gene for the TPO receptor, c-mpl, despite high levels of serum TPO ... There is a low risk of transformation to leukemia with this disorder. The primary treatment consists of anagrelide or ... Pang L, Weiss MJ, Poncz M (2005). "Megakaryocyte biology and related disorders". J. Clin. Invest. 115 (12): 3332-38. doi: ...
https://en.wikipedia.org/wiki/Megakaryocyte
Cancer in catsCancer in cats
Myeloproliferative tumors are types of genetic disorders passed through generations. It can affect the bone marrow, white and ... Similar symptoms occur in blood clotting disorders, they include weakness, labored breathing, pale muscus membranes and a loss ...
https://en.wikipedia.org/wiki/Cancer_in_cats
Janus kinase 2Janus kinase 2
Hsu HC (March 2007). "Pathogenetic role of JAK2 V617F mutation in chronic myeloproliferative disorders". Journal of the Chinese ... Berger R (May 2006). "[A recurrent mutation of the JAK2 gene in chronic myeloproliferative disorders]". Pathologie-Biologie. 54 ... and myelofibrosis as well as other myeloproliferative disorders. This mutation (V617F), a change of valine to phenylalanine at ... "A gain-of-function mutation of JAK2 in myeloproliferative disorders". The New England Journal of Medicine. 352 (17): 1779-90. ...
https://en.wikipedia.org/wiki/Janus_kinase_2
Denis AlexanderDenis Alexander
Inhibition of the Bcl-xL deamidation pathway in myeloproliferative disorders'. Beyond Science (1972), Oxford: Lion Publishing, ...
https://en.wikipedia.org/wiki/Denis_Alexander
Polycythemia veraPolycythemia vera
"Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet. 365 (9464): 1054-61. doi:10.1016/ ... Harrington, Jim (March 9, 2022). "'Gifted artist' Ron Miles dies of a rare blood disorder at 58". The Mercury News. Retrieved ... Polycythemia vera is an uncommon myeloproliferative neoplasm (a type of chronic leukemia) in which the bone marrow makes too ... and the related myeloproliferative disease essential thrombocythemia) is erythromelalgia. This is a burning pain in the hands ...
https://en.wikipedia.org/wiki/Polycythemia_vera
Chronic myelogenous leukemiaChronic myelogenous leukemia
Tefferi A (2006). "Classification, diagnosis and management of myeloproliferative disorders in the JAK2V617F era". Hematology. ... CML is a clonal bone marrow stem cell disorder in which a proliferation of mature granulocytes (neutrophils, eosinophils and ... It is a type of myeloproliferative neoplasm associated with a characteristic chromosomal translocation called the Philadelphia ...
https://en.wikipedia.org/wiki/Chronic_myelogenous_leukemia
Myeloproliferative neoplasmMyeloproliferative neoplasm
Myeloproliferative+Disorders at the US National Library of Medicine Medical Subject Headings (MeSH) MPN Info via Cancer.gov ( ... July 2008). "MPL mutations in myeloproliferative disorders: analysis of the PT-1 cohort". Blood. 112 (1): 141-9. doi:10.1182/ ... Myeloproliferative neoplasms (MPNs) are a group of rare blood cancers in which excess red blood cells, white blood cells or ... The concept of myeloproliferative disease was first proposed in 1951 by the hematologist William Dameshek. The discovery of the ...
https://en.wikipedia.org/wiki/Myeloproliferative_neoplasm
Hemoglobin variantsHemoglobin variants
Elevated Hemoglobin F levels are also associated with Leukemia and myeloproliferative disorders.[citation needed] Hemoglobin H ... Red blood cell disorders, Genetic disorders with no OMIM, Hemoglobins, Respiratory physiology). ... This rare condition is called Hereditary Persistence of Fetal Hemoglobin (HPFH). This is a group of disorders where the ...
https://en.wikipedia.org/wiki/Hemoglobin_variants
LL-100 panelLL-100 panel
"JAK2 V617F tyrosine kinase mutation in cell lines derived from myeloproliferative disorders". Leukemia. 20 (3): 471-6. doi: ...
https://en.wikipedia.org/wiki/LL-100_panel
FibronectinFibronectin
"Substance p-fibronectin-cytokine interactions in myeloproliferative disorders with bone marrow fibrosis". Acta Haematologica. ...
https://en.wikipedia.org/wiki/Fibronectin
Acute megakaryoblastic leukemiaAcute megakaryoblastic leukemia
"Transient myeloproliferative disorder in children with Down syndrome: clarity to this enigmatic disorder". British Journal of ... "Transient myeloproliferative disorder in neonates without Down syndrome: case report and review". European Journal of ... The disorder is far more fulminant than non-DS-AMKL and DS-AMKL and generally presents with more serious hematological symptoms ... However, non-DS-AMKL is a more aggressive and rapidly progressing disorder than DS-AMKL. Nonetheless, the presentation of non- ...
https://en.wikipedia.org/wiki/Acute_megakaryoblastic_leukemia
JAK-STAT signaling pathwayJAK-STAT signaling pathway
"JAK/STAT Pathways in Cytokine Signaling and Myeloproliferative Disorders: Approaches for Targeted Therapies". Genes & Cancer. 1 ... Mutations of the STAT5 protein, which can signal with JAK3, has been shown to result in autoimmune disorders. It has been ... Since excessive JAK-STAT signalling is responsible for some cancers and immune disorders, JAK inhibitors have been proposed as ... Patients with a faulty JAK-STAT signalling pathway may also experience skin disorders. For example, non-functional cytokine ...
https://en.wikipedia.org/wiki/JAK-STAT_signaling_pathway
Clonal hematopoiesisClonal hematopoiesis
... implications for the myeloproliferative disorders and myelodysplastic syndromes". British Journal of Haematology. 97 (4): 920- ... The acquisition of additional mutations can cause CHIP to transform into the related blood disorders MDS and AML. Acute myeloid ... Clonal hematopoiesis is sometimes compared to the unrelated blood disorders of monoclonal gammopathy of undetermined ... Hematopoietic stem cell Hematopoietic stem cell transplantation Hematology Myelodysplastic syndrome Myeloproliferative neoplasm ...
https://en.wikipedia.org/wiki/Clonal_hematopoiesis
Derald RuttenbergDerald Ruttenberg
It specializes in hematologic cancers such as leukemia, lymphoma, multiple myeloma, and myeloproliferative disorders. ...
https://en.wikipedia.org/wiki/Derald_Ruttenberg
Transient myeloproliferative diseaseTransient myeloproliferative disease
"Transient myeloproliferative disorder in children with Down syndrome: clarity to this enigmatic disorder". British Journal of ... Zipursky A, Brown EJ, Christensen H, Doyle J (March 1999). "Transient myeloproliferative disorder (transient leukemia) and ... and/or other signs or symptoms of the disorder. However, some individuals with transient myeloproliferative disease have a ... "Mutations in GATA1 in both transient myeloproliferative disorder and acute megakaryoblastic leukemia of Down syndrome". Blood ...
https://en.wikipedia.org/wiki/Transient_myeloproliferative_disease
Deaths in July 2006Deaths in July 2006
Winthrop Paul Rockefeller, 57, American billionaire and Lieutenant Governor of Arkansas since 1996, myeloproliferative disorder ...
https://en.wikipedia.org/wiki/Deaths_in_July_2006
Allen C EavesAllen C Eaves
Transient suppression of clonal hemopoiesis associated with pregnancy in a patient with a myeloproliferative disorder. J Clin ...
https://en.wikipedia.org/wiki/Allen_C_Eaves
Atypical chronic myeloid leukemiaAtypical chronic myeloid leukemia
It is a heterogeneous disorder belonging to the group of myelodysplastic/myeloproliferative (MDS/MPN) syndromes. In aCML many ...
https://en.wikipedia.org/wiki/Atypical_chronic_myeloid_leukemia
Yoram ChaiterYoram Chaiter
Chaiter Y, Brenner B, Aghai E, Tatarsky I. High incidence of myeloproliferative disorders in Ashkenazi Jews in northern Israel. ... thesis theme was Epidemiology of Myeloproliferative Disorders in North Israel. He was Head Physician of Recruitment Center from ...
https://en.wikipedia.org/wiki/Yoram_Chaiter
FIP1L1FIP1L1
While the success of Gleevec in treating the myeloproliferative neoplasm/myeloblastic leukemia or T-lymphoblastic leukemia/ ... Gotlib J (2015). "World Health Organization-defined eosinophilic disorders: 2015 update on diagnosis, risk stratification, and ... a type of myeloproliferative neoplasm/myeloblastic leukemia not distinguished by eosinophilia; or c) T-lymphoblastic leukemia/ ...
https://en.wikipedia.org/wiki/FIP1L1
Nodular regenerative hyperplasiaNodular regenerative hyperplasia
It is associated with rheumatoid arthritis, Felty syndrome, myeloproliferative disorders, hereditary hemorrhagic telangiectasia ...
https://en.wikipedia.org/wiki/Nodular_regenerative_hyperplasia
PolysomyPolysomy
... or myeloproliferative disorder (MPD) with a high incidence of secondary diseases and a six-month survival rate are associated ... RFLP also aids in the identification of the Huntingtin (HTT) gene which is predictive of an adult-onset autosomal disorder ... Some of the most frequent genetic disorders are abnormalities of sex chromosomes, but polysomies rarely occur. 49,XXXXY ... Ross, Helen L.; Elias, Sherman (1997). "Maternal Serum Screening for Fetal Genetic Disorders". Obstetrics and Gynecology ...
https://en.wikipedia.org/wiki/Polysomy
AI-10-49AI-10-49
Point-mutations in RUNX1 gene have been reported in patients with familial platelet disorder, myeloid dysplastic syndrome, and ... July 2005). "Loss of Runx1 perturbs adult hematopoiesis and is associated with a myeloproliferative phenotype". Blood. 106 (2 ... November 2001). "A novel CBFA2 single-nucleotide mutation in familial platelet disorder with propensity to develop myeloid ...
https://en.wikipedia.org/wiki/AI-10-49
MacrocytosisMacrocytosis
... the cause is bone marrow dysplasia secondary to alcohol use disorder. Poor absorption of vitamin B12 in the digestive tract can ... which bind on erythrocytes and induce membrane expansion myeloproliferative disease myelodysplastic syndrome which most ...
https://en.wikipedia.org/wiki/Macrocytosis
Tumors of the hematopoietic and lymphoid tissuesTumors of the hematopoietic and lymphoid tissues
Within this category, lymphomas are more common than leukemias.[citation needed] Myelodysplastic-myeloproliferative diseases ... Hodgkin lymphoma Immunodeficiency-associated lymphoproliferative disorders Post-transplant lymphoproliferative disorders (PTLD ... myelodysplastic syndromes and myeloproliferative diseases are myeloid in origin. A subgroup of them are more severe and are ... Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis Myelodysplastic/myeloproliferative ...
https://en.wikipedia.org/wiki/Tumors_of_the_hematopoietic_and_lymphoid_tissues
PDGFRBPDGFRB
Steer EJ, Cross NC (2002). "Myeloproliferative disorders with translocations of chromosome 5q31-35: role of the platelet- ... movement disorders, parkinsonism, seizures, headache) features and psychiatric (e.g. cognitive impairment, mood disorders, ... Other genetic abnormalities in PDGFRB lead to various forms of potentially malignant bone marrow disorders: small deletions in ... Soriano P (1994). "Abnormal kidney development and hematological disorders in PDGF beta-receptor mutant mice". Genes & ...
https://en.wikipedia.org/wiki/PDGFRB
Lactate dehydrogenase bLactate dehydrogenase b
"Association of an exonic LDHA polymorphism with altered respiratory response in probands at high risk for panic disorder". Am. ... "Increased serum lactate dehydrogenase isoenzymes in Ph-negative chronic myeloproliferative diseases: a metabolic adaptation?". ... among the sclerosing bone disorders". J. Bone Miner. Res. 25 (11): 2515-26. doi:10.1002/jbmr.130. PMID 20499337. S2CID 22022750 ...
https://en.wikipedia.org/wiki/Lactate_dehydrogenase_b
ErythromelalgiaErythromelalgia
Myeloproliferative disease Hypercholesterolemia Autoimmune disorder Small fiber peripheral neuropathy Fabry's disease Mercury ... Erythromelalgia may occur either as a primary or secondary disorder (i.e. a disorder in and of itself or a symptom of another ... often related to a myeloproliferative disorder and has also seen cases of: hypertension, diabetes mellitus, rheumatoid ... In 2004 erythromelalgia became the first human disorder in which it has been possible to associate an ion channel mutation with ...
https://en.wikipedia.org/wiki/Erythromelalgia
CyclophosphamideCyclophosphamide
Myeloproliferative neoplasms, including acute leukemia, non-Hodgkin lymphoma and multiple myeloma, occurred in 5 of 119 ... it can also occur with the lower doses used in the management of inflammatory disorders. Acrolein is toxic to the bladder ...
https://en.wikipedia.org/wiki/Cyclophosphamide
Bob Turner (Canadian politician)Bob Turner (Canadian politician)
He continues to practice medicine, with a special interest in blood disorders such as acute and chronic leukemias, lymphomas, ... myelodysplastic syndromes, myeloproliferative neoplasms, mast cell diseases and stem cell transplantation. In the past, Turner ...
https://en.wikipedia.org/wiki/Bob_Turner_(Canadian_politician)
Congenital amegakaryocytic thrombocytopeniaCongenital amegakaryocytic thrombocytopenia
The cause for this disorder appears to be a mutation in the gene for the TPO receptor, c-mpl, despite high levels of serum TPO ... Thrombopoietin Myeloproliferative leukemia virus oncogene Ballmaier M, Germeshausen M, Schulze H, et al. (January 2001). "c-mpl ... Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare inherited disorder. The primary manifestations are ...
https://en.wikipedia.org/wiki/Congenital_amegakaryocytic_thrombocytopenia
LestaurtinibLestaurtinib
JAK2/STAT5 signaling and the proliferation of primary erythroid cells from patients with myeloproliferative disorders". Blood. ...
https://en.wikipedia.org/wiki/Lestaurtinib
Janus kinase inhibitorJanus kinase inhibitor
Cucurbitacin I (JSI-124). CHZ868 - a type II JAK2 inhibitor for use in myeloproliferative disorders and chronic myelomonocytic ... against JAK1 and JAK2 for myeloproliferative disorders and relapsed/refractory metastatic pancreatic cancer. Pacritinib (SB1518 ... "FDA approves treatment for patients with rare bone marrow disorder". FDA. 16 August 2019. Retrieved 16 August 2019. "U.S. FDA ... "Pacritinib in Combination with Low Dose Decitabine in Intermediate-High Risk Myelofibrosis or Myeloproliferative Neoplasm (MPN ...
https://en.wikipedia.org/wiki/Janus_kinase_inhibitor
Medical genetics of JewsMedical genetics of Jews
"Ashkenazi Disorders: Mendelian - Mucolipidosis IV". The Chicago Center for Jewish Genetic Disorders. "Myeloproliferative ... "Ashkenazi Disorders: Mendelian - Niemann-Pick disease". The Chicago Center for Jewish Genetic Disorders. Boas FE (August 2000 ... "Ashkenazi Disorders: Mendelian - Familial dysautonomia". The Chicago Center for Jewish Genetic Disorders. about one in 30 ... "Ashkenazi Disorders: Mendelian - Non-Classical Adrenal Hyperplasia". Jewish Genetic Disorders Organization. Shalimar A, Sharaf ...
https://en.wikipedia.org/wiki/Medical_genetics_of_Jews
Myelophthisic anemiaMyelophthisic anemia
Other causes include myeloproliferative disorders (especially late-stage or spent polycythemia vera), granulomatous diseases, ... disordered metabolism related to the underlying disorder, and, in some cases, erythrophagocytosis. Some cases of myelophthisis ... Myelophthisis can occur in the setting of chronic myeloproliferative disease (e.g. myelofibrosis), leukemia, lymphoma, and ... Treatment of this disorder involves treatment of the underlying cancer. List of circulatory system conditions List of ...
https://en.wikipedia.org/wiki/Myelophthisic_anemia
GoutGout
... and myeloproliferative disorders such as polycythemia. A body mass index greater than or equal to 35 increases male risk of ... The rare genetic disorders familial juvenile hyperuricemic nephropathy, medullary cystic kidney disease, ... Gout is a disorder of purine metabolism, and occurs when its final metabolite, uric acid, crystallizes in the form of ...
https://en.wikipedia.org/wiki/Gout
Venous thrombosisVenous thrombosis
2012). "Risk of pulmonary embolism in patients with autoimmune disorders: a nationwide follow-up study from Sweden". Lancet. ... venous catheters Inflammatory diseases/some autoimmune diseases Nephrotic syndrome Obesity Infection HIV Myeloproliferative ...
https://en.wikipedia.org/wiki/Venous_thrombosis
Pernicious anemiaPernicious anemia
Normal serum levels may be found in cases of deficiency where myeloproliferative disorders, liver disease, transcobalamin II ... Other disorders that can disrupt the absorption of vitamin B12 in the small intestine include celiac disease, surgical removal ... They are found in about half of PA patients and are very rarely found in other disorders. These antibody tests can distinguish ... Parietal cell antibodies are found in other autoimmune disorders and also in up to 10% of healthy individuals. However, around ...
https://en.wikipedia.org/wiki/Pernicious_anemia
EmperipolesisEmperipolesis
... myeloproliferative disorders, myelodysplastic syndrome) Engulfment of inflammatory cells by histiocytes, which is a hallmark of ... Engulfment of hemapoietic cells by megakaryocytes such as in hematolymphoid disorders (Hodgkin's disease, leukemia, acute and ...
https://en.wikipedia.org/wiki/Emperipolesis
NeoplasmsPolycythemia veraNeoplasmMPNsMyelofibrosisMPDs8p11 myeloproliferative syndromeNeoplasticSyndromesClonalTransient myeloproliferativeHematologicDiseasesLeukemiaV617FLymphomaProteinsBoneComplicationsPlatelet FunctionDiseaseDiagnosisMalignancyPatientsJAK2TreatmentsCancersPosttraumatic stress dTranslocationEssentialMolecularProteinPrognosisGroup of disordersCoagulationSymptomsPulmonary
Neoplasms16
  • Myeloproliferative neoplasms, or MPNs - also called myeloproliferative disorders, or MPDs - are a collection of blood disorders that are believed to be caused by mutations in bone marrow stem cells. (ucsfhealth.org)
  • Myeloproliferative neoplasms (MPN), previously called myeloproliferative disorders, are diseases of the bone marrow and blood. (utahcancer.com)
  • Many times, especially in the early stages, myeloproliferative neoplasms (previously called myeloproliferative disorders) do not have symptoms. (utahcancer.com)
  • Myeloproliferative neoplasms, previously called myeloproliferative disorders, can be challenging to treat. (utahcancer.com)
  • Purpose: The objectives of this study were 1) to assess PV reporting to the PCR in 2006-2009, 2) to determine whether a cancer cluster persisted, and 3) to determine whether other myeloproliferative neoplasms (MPNs), including essential thrombocytopenia (ET), were subject to similar reporting problems. (cdc.gov)
  • Precapillary pulmonary hypertension (PH) implies a worse prognosis in myeloproliferative neoplasms (MPN). (archivesofmedicalscience.com)
  • A sub-group of myeloproliferative neoplasms (MPN) is BCR-ABL1-negative MPN that includes polycythemia vera, essential thrombocythemia, and primary myelofibrosis [ 1 ]. (archivesofmedicalscience.com)
  • Refer to the PDQ summary on Chronic Myeloproliferative Neoplasms Treatment for more information. (cancer.gov)
  • Polycythemia vera (PV) is one disease in a group of Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) and is characterized by erythrocytosis, uncontrolled and autonomous hematopoiesis, and evolution to end-stage myelofibrosis or acute nonlymphocytic leukemia. (cdc.gov)
  • What are myeloproliferative neoplasms (MPNs)? (voicesofmpn.com)
  • Myeloproliferative neoplasms (MY-ah-lo-pro-LIF-er-uh-tiv NEE-o-plaz-uhms), or MPNs, are a group of rare, chronic blood cancers in which a person's bone marrow does not function properly. (voicesofmpn.com)
  • Once known as myeloproliferative disorders (MPDs), myeloproliferative neoplasms were renamed and officially classified as cancers of the bone marrow in 2008 by the World Health Organization. (voicesofmpn.com)
  • Myeloproliferative neoplasms, or MPNs, are a group of rare, chronic blood cancers in which a person's bone marrow does not function properly. (voicesofmpn.com)
  • In particular, overactive JAK signalling is linked to the development of cancer-like conditions called myeloproliferative neoplasms (MPNs) - which include polycythemia vera, essential thrombocythemia and primary myelofibrosis - as well as certain acute childhood leukaemias . (edu.au)
  • RUX, a Janus kinase (JAK) 1 and 2 inhibitor that inhibits cytokine signaling and reduces symptoms in myelofibrosis, is increasingly used to treat myeloproliferative neoplasms (MPNs). (ajmc.com)
  • The MPN Research Foundation has a single goal: to stimulate original research in pursuit of new treatments - and eventually a cure - for polycythemia vera,essential thrombocythemia and myelofibrosis, known collectively as myeloproliferative neoplasms (MPNs). (mpnfoundation.org)
Polycythemia vera4
  • In an attempt to define the abnormality underlying the hemorrhagic diathesis, the platelet function of 21 consecutive patients admitted to the University of Minnesota Hospitals with various myeloproliferative disorders (polycythemia vera, myelogenous leukemia, agnogenic myeloid metaplasia, and erythroleukemia) was evaluated by the use of both the older, less sensitive methods, and those more sensitive and reliable techniques which have recently become available. (umn.edu)
  • The myeloproliferative disorder, polycythemia vera, and the rare congenital polycythemias represent primary erythrocytosis. (hindawi.com)
  • The differential diagnosis of CML includes leukemoid reaction, myeloproliferative disorders , essential thrombocythemia , and polycythemia vera with iron deficiency. (medscape.com)
  • A specific type of primary polycythemia, polycythemia rubra vera (often just called polycythemia vera) is an acquired myeloproliferative disorder which is discussed in detail elsewhere ( Pediatric Polycythemia Vera ). (medscape.com)
Neoplasm7
  • These symptoms do not always mean you have a myeloproliferative neoplasm. (utahcancer.com)
  • This cohort also includes patients with myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) overlap syndromes, excluding juvenile myelomonocytic leukemia (JMML). (clinicaltrials.gov)
  • Myelofibrosis (MF) is a rare chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)-negative myeloproliferative neoplasm characterized by progressive bone marrow fibrosis, inefficient hematopoiesis, and shortened survival. (dovepress.com)
  • Myelofibrosis (MF) is a chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)-negative stem cell myeloproliferative neoplasm (MPN) characterized by bone marrow fibrosis, ineffective hematopoiesis, extramedullary hematopoiesis (EMH), splenomegaly, shortened survival and progressive abdominal and constitutional symptoms, as well as other general chronic debilitating complaints. (dovepress.com)
  • in patients with a Philadelphia chromosome-negative myeloproliferative neoplasm (MPN). (cdc.gov)
  • A positive result is not specific for a particular subtype of myeloproliferative neoplasm and clinicopathologic correlation is necessary in all cases. (marshfieldlabs.org)
  • A negative result does not exclude the presence of a myeloproliferative neoplasm or other neoplastic process. (marshfieldlabs.org)
MPNs1
  • All of the MPNs are hematopoietic rates from 2001 (when MPNs first became reportable) stem cell disorders of common clonal heritage, character- through 2005 in these 3 counties. (cdc.gov)
Myelofibrosis3
  • IMSEAR at SEARO: Myelofibrosis in myeloproliferative disorders. (who.int)
  • Myelofibrosis in myeloproliferative disorders. (who.int)
  • mutational frequency is estimated at approximately 50% in both essential thrombocythemia (ET) and myelofibrosis (MF), up to 20% in certain subcategories of atypical myeloproliferative disorder (atypical MPD), less than 3% in de novo myelodysplastic syndrome (MDS) or acute myeloid leukemia, and 0% in chronic myeloid leukemia (CML). (ashpublications.org)
MPDs3
  • Background and Aims: Chronic myeloproliferative diseases (MPDs) are heterogenous group of haematological malignant disorders. (elsevier.com)
  • 5 The association of JAK2 V617F with myeloproliferative disorders (MPDs), including PV, ET, and MF, was first reported in 2005. (ashpublications.org)
  • 2 , - 5 Subsequently, the mutation has been demonstrated in other myeloid disorders including atypical MPDs and MDS 6 , 7 as well as acute leukemia cell lines including the human erythroleukemia (HEL) cell line. (ashpublications.org)
8p11 myeloproliferative syndrome2
  • A genetic change involving the ZMYM2 gene causes most cases of 8p11 myeloproliferative syndrome. (medlineplus.gov)
  • 8p11 myeloproliferative syndrome most commonly results from a rearrangement (translocation) of genetic material between chromosome 13 and chromosome 8. (medlineplus.gov)
Neoplastic2
  • Although they are not associated with neoplastic tissues, like other cancers, myeloproliferative disorders are classified within blood cancers. (petmd.com)
  • FeLV is transmitted horizontally among domestic cats through body secretions ( 6 ) and was the first retrovirus shown to cause both neoplastic and degenerative disorders ( 7 , 8 ). (cdc.gov)
Syndromes1
  • Ph1-negative CML is a poorly defined entity that is less clearly distinguished from other myeloproliferative syndromes. (cancer.gov)
Clonal4
  • The presence of JAK2 (V617F) mutation is considered an important criterion for the exclusion of secondary/reactive from clonal disorders. (elsevier.com)
  • These disorders are characterized by stem cell-derived clonal myeloproliferation. (archivesofmedicalscience.com)
  • CML is a clonal disorder that is usually easily diagnosed because the leukemic cells of more than 95% of patients have a distinctive cytogenetic abnormality, the Philadelphia chromosome (Ph1). (cancer.gov)
  • CML is a clonal myeloproliferative disorder of pluripotent stem cells. (medscape.com)
Transient myeloproliferative2
  • Acute megakaryoblastic leukaemia (AMKL) and transient myeloproliferative disorder (TMD) in Down syndrome: a multi-step model of myeloid leukaemogenesis. (ox.ac.uk)
  • Children with Down syndrome (DS) have a marked increase in susceptibility to Acute Megakaryoblastic Leukaemia (DS-AMKL) and the closely linked neonatal preleukaemic syndrome, Transient Myeloproliferative Disorder (DS-TMD). (ox.ac.uk)
Hematologic3
  • This identify comprehensively covers power myeloid leukemia and Ph-negative persistent myeloproliferative issues and is an important source for all practitioners in Hematologic Oncology. (mymarketusa.com)
  • Dr. Naik specializes in the treatment of adult patients with wide-array of non-malignant hematologic diseases, including autoimmune cytopenias, coagulation disorders, hemoglobinopathies, and myeloid disorders. (hopkinsmedicine.org)
  • Northwell Health intends to use IV-Cell for all relevant cytogenetics cases within their entire laboratory system, benefiting patients with leukemia, myelodysplasia, myeloproliferative disorders and other hematologic malignancies. (accesswire.com)
Diseases2
  • CML is one of a group of diseases called the myeloproliferative disorders. (cancer.gov)
  • It is difficult to differentiate myeloproliferative or myelodysplastic syndrome from CML owing to the presence of thrombocytosis, neutrophilia, and splenomegaly in both diseases. (medscape.com)
Leukemia1
  • The myeloproliferative disorder usually develops into another form of blood cancer called acute myeloid leukemia. (medlineplus.gov)
V617F2
  • Since the discovery of the JAK2 V617F mutation, the clinical and pathological consequences of this acquired defect have been extensively investigated to determine whether its presence characterises a distinct subgroup of myeloproliferative disorders (MPD). (nih.gov)
  • To date, JAK2 V617F has not been described in patients with reactive myeloproliferation, lymphoid disorders, or solid tumor. (ashpublications.org)
Lymphoma1
  • This condition is characterized by an increased number of white blood cells (myeloproliferative disorder) and the development of lymphoma, a blood-related cancer that causes tumor formation in the lymph nodes. (medlineplus.gov)
Proteins2
  • ZNF198 protein, involved in rearrangement in myeloproliferative disease, forms complexes with the DNA repair-associated HHR6A/6B and RAD18 proteins. (medlineplus.gov)
  • Development and application of novel immunoassays for eosinophil granule major basic proteins to evaluate eosinophilia and myeloproliferative disorders. (nih.gov)
Bone2
  • Myeloproliferative Disorders are a group of disorders that involve excess cell production originating from the bone marrow. (petmd.com)
  • Regular blood testing and bone marrow examination is recommended during treatment to determine the dog's response to the therapy and the progression of the disorder. (petmd.com)
Complications1
  • Despite extensive investigation of all aspects of the coagulation system, the mechanisms underlying the hemorrhagic and thrombotic complications seen in the "myeloproliferative disorders" have not been satisfactorily elucidated. (umn.edu)
Platelet Function1
  • Conditions associated with decreased platelet aggregation include suspected hereditary and acquired disorders of platelet function. (medscape.com)
Disease3
  • A distinction is generally made between primary (idiopathic or genetic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders ), as well as between early- and late-onset disease. (medscape.com)
  • ZNF198, a zinc finger protein rearranged in myeloproliferative disease, localizes to the PML nuclear bodies and interacts with SUMO-1 and PML. (medlineplus.gov)
  • At the meeting, representatives from ATSDR, the Centers for Disease Control and Prevention, the Pennsylvania Department of Health, the Pennsylvania Department of Environmental Protection, the Mount Sinai School of Medicine, the Geisinger Clinic, and the Myeloproliferative Disease (MPD) Research Consortium will present overviews of their current PV research projects. (cdc.gov)
Diagnosis3
  • Shomali W, Gotlib J. World Health Organization-defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management. (medscape.com)
  • You will gain an understanding of blood cell formation, structure and function as well as learning about haematological disorders and their diagnosis and treatment. (manchester.ac.uk)
  • This unit is aimed at providing an extensive knowledge of the main areas of haematology including understanding, diagnosis and treatment of haematological disorders and to introduce the main areas of interest in blood transfusion. (manchester.ac.uk)
Malignancy1
  • To determine the cause of renal infarction, investigations encompass extensive cardiac workup (echocardiography, prolonged cardiac monitoring, and blood cultures), investigations for hypercoagulable states ( antiphospholipid syndrome , hyperhomocysteinemia, paroxysmal nocturnal hemoglobinuria , and myeloproliferative disorders), appropriate malignancy screening for the age of the patient, and collagen disorder testing. (medscape.com)
Patients2
  • With the recent type of persistent myeloproliferative issues, and the increase of curiosity in molecularly precise remedies, this well timed textual content brings jointly foreign specialists at the subject to debate the present applied sciences and their implications for the therapy of patients. (mymarketusa.com)
  • Because a significant proportion of BCS patients are found to have an underlying myeloproliferative disorder (MPD), antiplatelet therapy may be a more rational treatment strategy for this subgroup. (uab.edu)
JAK22
  • Todas ellas producen alteraciones en la regulación de las CÉLULAS PROGENITORAS MIELOIDES, muy frecuentemente por causa de una mutación en la PROTEINA TIROSINA QUINASA JAK2. (bvsalud.org)
  • Many experts believe people with PV and related blood disorders may test positive for the JAK2 marker for a number of years before ever exhibiting symptoms of PV. (cdc.gov)
Treatments3
  • UCSF is dedicated to delivering the most advanced treatments for myeloproliferative disorders with care and compassion. (ucsfhealth.org)
  • We are also dedicated to discovering better treatments for myeloproliferative disorders through research. (ucsfhealth.org)
  • Treatments depend on the disorder and how severe it is. (medlineplus.gov)
Cancers1
  • During the course of your care, it is important to see a doctor who specializes in treating blood disorders or blood cancers. (voicesofmpn.com)
Posttraumatic stress d1
  • Military-related posttraumatic stress disorder and mindfulness meditation. (greenmedinfo.com)
Translocation1
  • parietal translocation and parents with chromosomal disorders 3 . (bvsalud.org)
Essential1
  • We describe a case of BPDCN in a 65-year-old female patient with myeloproliferative disorder (essential thrombocythemia) and chronic lymphocytic leukaemia. (bmj.com)
Molecular2
  • Molecular biology may provide the key to understanding this disorder. (medscape.com)
  • Eosinophils and eosinophil-associated disorders: immunological, clinical, and molecular complexity. (medscape.com)
Protein1
  • 3 ] This, in turn, results in a fused BCR/ABL gene and in the production of an abnormal tyrosine kinase protein that causes the disordered myelopoiesis found in CML. (cancer.gov)
Prognosis1
  • Unfortunately, the prognosis of dogs suffering from these disorders is poor. (petmd.com)
Group of disorders1
  • Pulmonary hypertension represents a heterogeneous group of disorders that are classified based on differences in clinical, hemodynamic, and histopathologic features. (archivesofmedicalscience.com)
Coagulation1
Symptoms1
  • There are several types of myeloproliferative disorders, and the best therapy depends on the type and the patient's symptoms. (ucsfhealth.org)
Pulmonary1
  • Pulmonary arterial hypertension (PAH - Group 1) is a progressive disorder characterized by remodelling of the small pulmonary arteries, resulting in increased pulmonary vascular resistance [ 10 ]. (archivesofmedicalscience.com)