Anemia: A reduction in the number of circulating ERYTHROCYTES or in the quantity of HEMOGLOBIN.Anemia, Aplastic: A form of anemia in which the bone marrow fails to produce adequate numbers of peripheral blood elements.Anemia, Hemolytic: A condition of inadequate circulating red blood cells (ANEMIA) or insufficient HEMOGLOBIN due to premature destruction of red blood cells (ERYTHROCYTES).Anemia, Refractory: A severe sometimes chronic anemia, usually macrocytic in type, that does not respond to ordinary antianemic therapy.Fanconi Anemia: Congenital disorder affecting all bone marrow elements, resulting in ANEMIA; LEUKOPENIA; and THROMBOPENIA, and associated with cardiac, renal, and limb malformations as well as dermal pigmentary changes. Spontaneous CHROMOSOME BREAKAGE is a feature of this disease along with predisposition to LEUKEMIA. There are at least 7 complementation groups in Fanconi anemia: FANCA, FANCB, FANCC, FANCD1, FANCD2, FANCE, FANCF, FANCG, and FANCL. (from Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=227650, August 20, 2004)Anemia, Hemolytic, Autoimmune: Acquired hemolytic anemia due to the presence of AUTOANTIBODIES which agglutinate or lyse the patient's own RED BLOOD CELLS.Anemia, Hypochromic: Anemia characterized by a decrease in the ratio of the weight of hemoglobin to the volume of the erythrocyte, i.e., the mean corpuscular hemoglobin concentration is less than normal. The individual cells contain less hemoglobin than they could have under optimal conditions. Hypochromic anemia may be caused by iron deficiency from a low iron intake, diminished iron absorption, or excessive iron loss. It can also be caused by infections or other diseases, therapeutic drugs, lead poisoning, and other conditions. (Stedman, 25th ed; from Miale, Laboratory Medicine: Hematology, 6th ed, p393)Anemia, Macrocytic: Anemia characterized by larger than normal erythrocytes, increased mean corpuscular volume (MCV) and increased mean corpuscular hemoglobin (MCH).Anemia, Pernicious: A megaloblastic anemia occurring in children but more commonly in later life, characterized by histamine-fast achlorhydria, in which the laboratory and clinical manifestations are based on malabsorption of vitamin B 12 due to a failure of the gastric mucosa to secrete adequate and potent intrinsic factor. (Dorland, 27th ed)Anemia, Sideroblastic: Anemia characterized by the presence of erythroblasts containing excessive deposits of iron in the marrow.Anemia, Sickle Cell: A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.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.Refractory Period, Electrophysiological: The period of time following the triggering of an ACTION POTENTIAL when the CELL MEMBRANE has changed to an unexcitable state and is gradually restored to the resting (excitable) state. During the absolute refractory period no other stimulus can trigger a response. This is followed by the relative refractory period during which the cell gradually becomes more excitable and the stronger impulse that is required to illicit a response gradually lessens to that required during the resting state.Anemia, Megaloblastic: A disorder characterized by the presence of ANEMIA, abnormally large red blood cells (megalocytes or macrocytes), and MEGALOBLASTS.Hemoglobins: The oxygen-carrying proteins of ERYTHROCYTES. They are found in all vertebrates and some invertebrates. The number of globin subunits in the hemoglobin quaternary structure differs between species. Structures range from monomeric to a variety of multimeric arrangements.Infectious Anemia Virus, Equine: A species of LENTIVIRUS, subgenus equine lentiviruses (LENTIVIRUSES, EQUINE), causing acute and chronic infection in horses. It is transmitted mechanically by biting flies, mosquitoes, and midges, and iatrogenically through unsterilized equipment. Chronic infection often consists of acute episodes with remissions.Anemia, Hemolytic, Congenital: Hemolytic anemia due to various intrinsic defects of the erythrocyte.Treatment Outcome: Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.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.Equine Infectious Anemia: Viral disease of horses caused by the equine infectious anemia virus (EIAV; INFECTIOUS ANEMIA VIRUS, EQUINE). It is characterized by intermittent fever, weakness, and anemia. Chronic infection consists of acute episodes with remissions.Chicken anemia virus: The type species of GYROVIRUS, a small, non-enveloped DNA virus originally isolated from contaminated vaccines in Japan. It causes chicken infectious anemia and may possibly play a key role in hemorrhagic anemia syndrome, anemia dermatitis, and blue wing disease.Anemia, Dyserythropoietic, Congenital: A familial disorder characterized by ANEMIA with multinuclear ERYTHROBLASTS, karyorrhexis, asynchrony of nuclear and cytoplasmic maturation, and various nuclear abnormalities of bone marrow erythrocyte precursors (ERYTHROID PRECURSOR CELLS). Type II is the most common of the 3 types; it is often referred to as HEMPAS, based on the Hereditary Erythroblast Multinuclearity with Positive Acidified Serum test.Anemia, Diamond-Blackfan: A rare congenital hypoplastic anemia that usually presents early in infancy. The disease is characterized by a moderate to severe macrocytic anemia, occasional neutropenia or thrombocytosis, a normocellular bone marrow with erythroid hypoplasia, and an increased risk of developing leukemia. (Curr Opin Hematol 2000 Mar;7(2):85-94)Fanconi Anemia Complementation Group Proteins: A diverse group of proteins whose genetic MUTATIONS have been associated with the chromosomal instability syndrome FANCONI ANEMIA. Many of these proteins play important roles in protecting CELLS against OXIDATIVE STRESS.Iron: A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.Salvage Therapy: A therapeutic approach, involving chemotherapy, radiation therapy, or surgery, after initial regimens have failed to lead to improvement in a patient's condition. Salvage therapy is most often used for neoplastic diseases.Recurrence: The return of a sign, symptom, or disease after a remission.Pregnancy Complications, Hematologic: The co-occurrence of pregnancy and a blood disease (HEMATOLOGIC DISEASES) which involves BLOOD CELLS or COAGULATION FACTORS. The hematologic disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS.Anemia, Neonatal: The mildest form of erythroblastosis fetalis in which anemia is the chief manifestation.Hematinics: Agents which improve the quality of the blood, increasing the hemoglobin level and the number of erythrocytes. They are used in the treatment of anemias.Drug Administration Schedule: Time schedule for administration of a drug in order to achieve optimum effectiveness and convenience.Hematocrit: The volume of packed RED BLOOD CELLS in a blood specimen. The volume is measured by centrifugation in a tube with graduated markings, or with automated blood cell counters. It is an indicator of erythrocyte status in disease. For example, ANEMIA shows a low value; POLYCYTHEMIA, a high value.Fanconi Anemia Complementation Group C Protein: A Fanconi anemia complementation group protein that regulates the activities of CYTOCHROME P450 REDUCTASE and GLUTATHIONE S-TRANSFERASE. It is found predominately in the CYTOPLASM, but moves to the CELL NUCLEUS in response to FANCE PROTEIN.Fanconi Anemia Complementation Group D2 Protein: A Fanconi anemia complementation group protein that undergoes mono-ubiquitination by FANCL PROTEIN in response to DNA DAMAGE. Also, in response to IONIZING RADIATION it can undergo PHOSPHORYLATION by ataxia telangiectasia mutated protein. Modified FANCD2 interacts with BRCA2 PROTEIN in a stable complex with CHROMATIN, and it is involved in DNA REPAIR by homologous RECOMBINATION.Fanconi Anemia Complementation Group A Protein: A Fanconi anemia complementation group protein that is the most commonly mutated protein in FANCONI ANEMIA. It undergoes PHOSPHORYLATION by PROTEIN KINASE B and forms a complex with FANCC PROTEIN in the CELL NUCLEUS.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.Antineoplastic Combined Chemotherapy Protocols: The use of two or more chemicals simultaneously or sequentially in the drug therapy of neoplasms. The drugs need not be in the same dosage form.Ferritins: Iron-containing proteins that are widely distributed in animals, plants, and microorganisms. Their major function is to store IRON in a nontoxic bioavailable form. Each ferritin molecule consists of ferric iron in a hollow protein shell (APOFERRITINS) made of 24 subunits of various sequences depending on the species and tissue types.Anemia, Hemolytic, Congenital Nonspherocytic: Any one of a group of congenital hemolytic anemias in which there is no abnormal hemoglobin or spherocytosis and in which there is a defect of glycolysis in the erythrocyte. Common causes include deficiencies in GLUCOSE-6-PHOSPHATE ISOMERASE; PYRUVATE KINASE; and GLUCOSE-6-PHOSPHATE DEHYDROGENASE.Pallor: A clinical manifestation consisting of an unnatural paleness of the skin.Erythrocyte Count: The number of RED BLOOD CELLS per unit volume in a sample of venous BLOOD.Antineoplastic Agents: Substances that inhibit or prevent the proliferation of NEOPLASMS.Erythrocyte Indices: ERYTHROCYTE size and HEMOGLOBIN content or concentration, usually derived from ERYTHROCYTE COUNT; BLOOD hemoglobin concentration; and HEMATOCRIT. The indices include the mean corpuscular volume (MCV), the mean corpuscular hemoglobin (MCH), and the mean corpuscular hemoglobin concentration (MCHC).Blood Transfusion: The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed)Hemoglobinometry: Measurement of hemoglobin concentration in blood.Drug Resistance, Neoplasm: Resistance or diminished response of a neoplasm to an antineoplastic agent in humans, animals, or cell or tissue cultures.Retrospective Studies: Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.Antibodies, Monoclonal, Murine-Derived: Antibodies obtained from a single clone of cells grown in mice or rats.Remission Induction: Therapeutic act or process that initiates a response to a complete or partial remission level.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.Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.Thrombocytopenia: A subnormal level of BLOOD PLATELETS.Follow-Up Studies: Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.Infusions, Intravenous: The long-term (minutes to hours) administration of a fluid into the vein through venipuncture, either by letting the fluid flow by gravity or by pumping it.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Thalidomide: A piperidinyl isoindole originally introduced as a non-barbiturate hypnotic, but withdrawn from the market due to teratogenic effects. It has been reintroduced and used for a number of immunological and inflammatory disorders. Thalidomide displays immunosuppressive and anti-angiogenic activity. It inhibits release of TUMOR NECROSIS FACTOR-ALPHA from monocytes, and modulates other cytokine action.Fanconi Anemia Complementation Group G Protein: A Fanconi anemia complementation group protein that undergoes PHOSPHORYLATION by CDC2 PROTEIN KINASE during MITOSIS. It forms a complex with other FANCONI ANEMIA PROTEINS and helps protect CELLS from DNA DAMAGE by genotoxic agents.Drug Resistance: Diminished or failed response of an organism, disease or tissue to the intended effectiveness of a chemical or drug. It should be differentiated from DRUG TOLERANCE which is the progressive diminution of the susceptibility of a human or animal to the effects of a drug, as a result of continued administration.Coombs Test: A test to detect non-agglutinating ANTIBODIES against ERYTHROCYTES by use of anti-antibodies (the Coombs' reagent.) The direct test is applied to freshly drawn blood to detect antibody bound to circulating red cells. The indirect test is applied to serum to detect the presence of antibodies that can bind to red blood cells.Reticulocyte Count: The number of RETICULOCYTES per unit volume of BLOOD. The values are expressed as a percentage of the ERYTHROCYTE COUNT or in the form of an index ("corrected reticulocyte index"), which attempts to account for the number of circulating erythrocytes.Immunosuppressive Agents: Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging.Dose-Response Relationship, Drug: The relationship between the dose of an administered drug and the response of the organism to the drug.Treatment Failure: A measure of the quality of health care by assessment of unsuccessful results of management and procedures used in combating disease, in individual cases or series.Iron, Dietary: Iron or iron compounds used in foods or as food. Dietary iron is important in oxygen transport and the synthesis of the iron-porphyrin proteins hemoglobin, myoglobin, cytochromes, and cytochrome oxidase. Insufficient amounts of dietary iron can lead to iron-deficiency anemia.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)Antibodies, Monoclonal: Antibodies produced by a single clone of cells.Cytarabine: A pyrimidine nucleoside analog that is used mainly in the treatment of leukemia, especially acute non-lymphoblastic leukemia. Cytarabine is an antimetabolite antineoplastic agent that inhibits the synthesis of DNA. Its actions are specific for the S phase of the cell cycle. It also has antiviral and immunosuppressant properties. (From Martindale, The Extra Pharmacopoeia, 30th ed, p472)Anticonvulsants: Drugs used to prevent SEIZURES or reduce their severity.Drug Therapy, Combination: Therapy with two or more separate preparations given for a combined effect.Prospective Studies: Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group.Iron Compounds: Organic and inorganic compounds that contain iron as an integral part of the molecule.Hepcidins: Forms of hepcidin, a cationic amphipathic peptide synthesized in the liver as a prepropeptide which is first processed into prohepcidin and then into the biologically active hepcidin forms, including in human the 20-, 22-, and 25-amino acid residue peptide forms. Hepcidin acts as a homeostatic regulators of iron metabolism and also possesses antimicrobial activity.Neoplasms: New abnormal growth of tissue. Malignant neoplasms show a greater degree of anaplasia and have the properties of invasion and metastasis, compared to benign neoplasms.Etoposide: A semisynthetic derivative of PODOPHYLLOTOXIN that exhibits antitumor activity. Etoposide inhibits DNA synthesis by forming a complex with topoisomerase II and DNA. This complex induces breaks in double stranded DNA and prevents repair by topoisomerase II binding. Accumulated breaks in DNA prevent entry into the mitotic phase of cell division, and lead to cell death. Etoposide acts primarily in the G2 and S phases of the cell cycle.Erythrocytes, Abnormal: Oxygen-carrying RED BLOOD CELLS in mammalian blood that are abnormal in structure or function.Boronic Acids: Inorganic or organic compounds that contain the basic structure RB(OH)2.PyrazinesMaximum Tolerated Dose: The highest dose of a biologically active agent given during a chronic study that will not reduce longevity from effects other than carcinogenicity. (from Lewis Dictionary of Toxicology, 1st ed)Erythrocyte Transfusion: The transfer of erythrocytes from a donor to a recipient or reinfusion to the donor.Drug Evaluation: Any process by which toxicity, metabolism, absorption, elimination, preferred route of administration, safe dosage range, etc., for a drug or group of drugs is determined through clinical assessment in humans or veterinary animals.Refractory Period, Psychological: A delayed response interval occurring when two stimuli are presented in close succession.Multiple Myeloma: A malignancy of mature PLASMA CELLS engaging in monoclonal immunoglobulin production. It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. Other features include ANEMIA; HYPERCALCEMIA; and RENAL INSUFFICIENCY.Survival Analysis: A class of statistical procedures for estimating the survival function (function of time, starting with a population 100% well at a given time and providing the percentage of the population still well at later times). The survival analysis is then used for making inferences about the effects of treatments, prognostic factors, exposures, and other covariates on the function.Prevalence: The total number of cases of a given disease in a specified population at a designated time. It is differentiated from INCIDENCE, which refers to the number of new cases in the population at a given time.Epilepsy: A disorder characterized by recurrent episodes of paroxysmal brain dysfunction due to a sudden, disorderly, and excessive neuronal discharge. Epilepsy classification systems are generally based upon: (1) clinical features of the seizure episodes (e.g., motor seizure), (2) etiology (e.g., post-traumatic), (3) anatomic site of seizure origin (e.g., frontal lobe seizure), (4) tendency to spread to other structures in the brain, and (5) temporal patterns (e.g., nocturnal epilepsy). (From Adams et al., Principles of Neurology, 6th ed, p313)Pancytopenia: Deficiency of all three cell elements of the blood, erythrocytes, leukocytes and platelets.Cyclophosphamide: Precursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the LIVER to form the active aldophosphamide. It has been used in the treatment of LYMPHOMA and LEUKEMIA. Its side effect, ALOPECIA, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer.Disease-Free Survival: Period after successful treatment in which there is no appearance of the symptoms or effects of the disease.Lymphoma, Non-Hodgkin: Any of a group of malignant tumors of lymphoid tissue that differ from HODGKIN DISEASE, being more heterogeneous with respect to malignant cell lineage, clinical course, prognosis, and therapy. The only common feature among these tumors is the absence of giant REED-STERNBERG CELLS, a characteristic of Hodgkin's disease.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.Vitamin B 12 Deficiency: A nutritional condition produced by a deficiency of VITAMIN B 12 in the diet, characterized by megaloblastic anemia. Since vitamin B 12 is not present in plants, humans have obtained their supply from animal products, from multivitamin supplements in the form of pills, and as additives to food preparations. A wide variety of neuropsychiatric abnormalities is also seen in vitamin B 12 deficiency and appears to be due to an undefined defect involving myelin synthesis. (From Cecil Textbook of Medicine, 19th ed, p848)Recombinant Proteins: Proteins prepared by recombinant DNA technology.Erythrocyte Aging: The senescence of RED BLOOD CELLS. Lacking the organelles that make protein synthesis possible, the mature erythrocyte is incapable of self-repair, reproduction, and carrying out certain functions performed by other cells. This limits the average life span of an erythrocyte to 120 days.Severity of Illness Index: Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.Transplantation, Autologous: Transplantation of an individual's own tissue from one site to another site.Hodgkin Disease: A malignant disease characterized by progressive enlargement of the lymph nodes, spleen, and general lymphoid tissue. In the classical variant, giant usually multinucleate Hodgkin's and REED-STERNBERG CELLS are present; in the nodular lymphocyte predominant variant, lymphocytic and histiocytic cells are seen.Hematopoietic Stem Cell Transplantation: Transfer of HEMATOPOIETIC STEM CELLS from BONE MARROW or BLOOD between individuals within the same species (TRANSPLANTATION, HOMOLOGOUS) or transfer within the same individual (TRANSPLANTATION, AUTOLOGOUS). Hematopoietic stem cell transplantation has been used as an alternative to BONE MARROW TRANSPLANTATION in the treatment of a variety of neoplasms.Vidarabine: A nucleoside antibiotic isolated from Streptomyces antibioticus. It has some antineoplastic properties and has broad spectrum activity against DNA viruses in cell cultures and significant antiviral activity against infections caused by a variety of viruses such as the herpes viruses, the VACCINIA VIRUS and varicella zoster virus.Survival Rate: The proportion of survivors in a group, e.g., of patients, studied and followed over a period, or the proportion of persons in a specified group alive at the beginning of a time interval who survive to the end of the interval. It is often studied using life table methods.Hematologic Diseases: Disorders of the blood and blood forming tissues.Risk Factors: An aspect of personal behavior or lifestyle, environmental exposure, or inborn or inherited characteristic, which, on the basis of epidemiologic evidence, is known to be associated with a health-related condition considered important to prevent.beta-Thalassemia: A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent.Neoplasm Recurrence, Local: The local recurrence of a neoplasm following treatment. It arises from microscopic cells of the original neoplasm that have escaped therapeutic intervention and later become clinically visible at the original site.Isavirus: A genus in the family ORTHOMYXOVIRIDAE containing one species: Infectious salmon anemia virus.Combined Modality Therapy: The treatment of a disease or condition by several different means simultaneously or sequentially. Chemoimmunotherapy, RADIOIMMUNOTHERAPY, chemoradiotherapy, cryochemotherapy, and SALVAGE THERAPY are seen most frequently, but their combinations with each other and surgery are also used.Prognosis: A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.Antilymphocyte Serum: Serum containing GAMMA-GLOBULINS which are antibodies for lymphocyte ANTIGENS. It is used both as a test for HISTOCOMPATIBILITY and therapeutically in TRANSPLANTATION.Administration, Oral: The giving of drugs, chemicals, or other substances by mouth.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.Pregnancy: The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.Malaria: A protozoan disease caused in humans by four species of the PLASMODIUM genus: PLASMODIUM FALCIPARUM; PLASMODIUM VIVAX; PLASMODIUM OVALE; and PLASMODIUM MALARIAE; and transmitted by the bite of an infected female mosquito of the genus ANOPHELES. Malaria is endemic in parts of Asia, Africa, Central and South America, Oceania, and certain Caribbean islands. It is characterized by extreme exhaustion associated with paroxysms of high FEVER; SWEATING; shaking CHILLS; and ANEMIA. Malaria in ANIMALS is caused by other species of plasmodia.Splenectomy: Surgical procedure involving either partial or entire removal of the spleen.Pain, Intractable: Persistent pain that is refractory to some or all forms of treatment.Arabinonucleosides: Nucleosides containing arabinose as their sugar moiety.Neutropenia: A decrease in the number of NEUTROPHILS found in the blood.Thalassemia: A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia.Splenomegaly: Enlargement of the spleen.Fanconi Anemia Complementation Group F Protein: A Fanconi anemia complementation group protein. It is an essential component of a nuclear core complex that protects the GENOME against CHROMOSOMAL INSTABILITY. It interacts directly with FANCG PROTEIN and helps stabilize a complex with FANCA PROTEIN and FANCC PROTEIN.Phenylhydrazines: Diazo derivatives of aniline, used as a reagent for sugars, ketones, and aldehydes. (Dorland, 28th ed)Fanconi Anemia Complementation Group E Protein: A Fanconi anemia complementation group protein that interacts with FANCC PROTEIN and FANCD2 PROTEIN. It promotes the accumulation of FANCC protein in the CELL NUCLEUS.Fetal Hemoglobin: The major component of hemoglobin in the fetus. This HEMOGLOBIN has two alpha and two gamma polypeptide subunits in comparison to normal adult hemoglobin, which has two alpha and two beta polypeptide subunits. Fetal hemoglobin concentrations can be elevated (usually above 0.5%) in children and adults affected by LEUKEMIA and several types of ANEMIA.Epilepsies, Partial: Conditions characterized by recurrent paroxysmal neuronal discharges which arise from a focal region of the brain. Partial seizures are divided into simple and complex, depending on whether consciousness is unaltered (simple partial seizure) or disturbed (complex partial seizure). Both types may feature a wide variety of motor, sensory, and autonomic symptoms. Partial seizures may be classified by associated clinical features or anatomic location of the seizure focus. A secondary generalized seizure refers to a partial seizure that spreads to involve the brain diffusely. (From Adams et al., Principles of Neurology, 6th ed, pp317)Mitomycin: An antineoplastic antibiotic produced by Streptomyces caespitosus. It is one of the bi- or tri-functional ALKYLATING AGENTS causing cross-linking of DNA and inhibition of DNA synthesis.Heart Conduction System: An impulse-conducting system composed of modified cardiac muscle, having the power of spontaneous rhythmicity and conduction more highly developed than the rest of the heart.Kidney Failure, Chronic: The end-stage of CHRONIC RENAL INSUFFICIENCY. It is characterized by the severe irreversible kidney damage (as measured by the level of PROTEINURIA) and the reduction in GLOMERULAR FILTRATION RATE to less than 15 ml per min (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002). These patients generally require HEMODIALYSIS or KIDNEY TRANSPLANTATION.Thrombocytosis: Increased numbers of platelets in the peripheral blood. (Dorland, 27th ed)Piracetam: A compound suggested to be both a nootropic and a neuroprotective agent.Folic Acid Deficiency: A nutritional condition produced by a deficiency of FOLIC ACID in the diet. Many plant and animal tissues contain folic acid, abundant in green leafy vegetables, yeast, liver, and mushrooms but destroyed by long-term cooking. Alcohol interferes with its intermediate metabolism and absorption. Folic acid deficiency may develop in long-term anticonvulsant therapy or with use of oral contraceptives. This deficiency causes anemia, macrocytic anemia, and megaloblastic anemia. It is indistinguishable from vitamin B 12 deficiency in peripheral blood and bone marrow findings, but the neurologic lesions seen in B 12 deficiency do not occur. (Merck Manual, 16th ed)Acute Disease: Disease having a short and relatively severe course.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)Bone Marrow DiseasesPurpura, Thrombocytopenic, Idiopathic: Thrombocytopenia occurring in the absence of toxic exposure or a disease associated with decreased platelets. It is mediated by immune mechanisms, in most cases IMMUNOGLOBULIN G autoantibodies which attach to platelets and subsequently undergo destruction by macrophages. The disease is seen in acute (affecting children) and chronic (adult) forms.Vitamin B 12: A cobalt-containing coordination compound produced by intestinal micro-organisms and found also in soil and water. Higher plants do not concentrate vitamin B 12 from the soil and so are a poor source of the substance as compared with animal tissues. INTRINSIC FACTOR is important for the assimilation of vitamin B 12.Horses: Large, hoofed mammals of the family EQUIDAE. Horses are active day and night with most of the day spent seeking and consuming food. Feeding peaks occur in the early morning and late afternoon, and there are several daily periods of rest.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.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.Transplantation, Homologous: Transplantation between individuals of the same species. Usually refers to genetically disparate individuals in contradistinction to isogeneic transplantation for genetically identical individuals.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.Infant, Newborn: An infant during the first month after birth.Ascites: Accumulation or retention of free fluid within the peritoneal cavity.Retreatment: The therapy of the same disease in a patient, with the same agent or procedure repeated after initial treatment, or with an additional or alternate measure or follow-up. It does not include therapy which requires more than one administration of a therapeutic agent or regimen. Retreatment is often used with reference to a different modality when the original one was inadequate, harmful, or unsuccessful.Atrioventricular Node: A small nodular mass of specialized muscle fibers located in the interatrial septum near the opening of the coronary sinus. It gives rise to the atrioventricular bundle of the conduction system of the heart.Platelet Count: The number of PLATELETS per unit volume in a sample of venous BLOOD.Erythroblasts: Immature, nucleated ERYTHROCYTES occupying the stage of ERYTHROPOIESIS that follows formation of ERYTHROID PRECURSOR CELLS and precedes formation of RETICULOCYTES. The normal series is called normoblasts. Cells called MEGALOBLASTS are a pathologic series of erythroblasts.Fatal Outcome: Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.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.Anti-Arrhythmia Agents: Agents used for the treatment or prevention of cardiac arrhythmias. They may affect the polarization-repolarization phase of the action potential, its excitability or refractoriness, or impulse conduction or membrane responsiveness within cardiac fibers. Anti-arrhythmia agents are often classed into four main groups according to their mechanism of action: sodium channel blockade, beta-adrenergic blockade, repolarization prolongation, or calcium channel blockade.Receptors, Transferrin: Membrane glycoproteins found in high concentrations on iron-utilizing cells. They specifically bind iron-bearing transferrin, are endocytosed with its ligand and then returned to the cell surface where transferrin without its iron is released.Osmotic Fragility: RED BLOOD CELL sensitivity to change in OSMOTIC PRESSURE. When exposed to a hypotonic concentration of sodium in a solution, red cells take in more water, swell until the capacity of the cell membrane is exceeded, and burst.Reticulocytes: Immature ERYTHROCYTES. In humans, these are ERYTHROID CELLS that have just undergone extrusion of their CELL NUCLEUS. They still contain some organelles that gradually decrease in number as the cells mature. RIBOSOMES are last to disappear. Certain staining techniques cause components of the ribosomes to precipitate into characteristic "reticulum" (not the same as the ENDOPLASMIC RETICULUM), hence the name reticulocytes.Cardiac Pacing, Artificial: Regulation of the rate of contraction of the heart muscles by an artificial pacemaker.Nitrogen Mustard Compounds: A group of alkylating agents derived from mustard gas, with the sulfur replaced by nitrogen. They were formerly used as toxicants and vesicants, but now function as antineoplastic agents. These compounds are also powerful mutagens, teratogens, immunosuppressants, and carcinogens.Parasitemia: The presence of parasites (especially malarial parasites) in the blood. (Dorland, 27th ed)Immunologic Factors: Biologically active substances whose activities affect or play a role in the functioning of the immune system.Ferrous Compounds: Inorganic or organic compounds that contain divalent iron.Iron Overload: An excessive accumulation of iron in the body due to a greater than normal absorption of iron from the gastrointestinal tract or from parenteral injection. This may arise from idiopathic hemochromatosis, excessive iron intake, chronic alcoholism, certain types of refractory anemia, or transfusional hemosiderosis. (From Churchill's Illustrated Medical Dictionary, 1989)LeukopeniaHeinz Bodies: Abnormal intracellular inclusions, composed of denatured hemoglobin, found on the membrane of red blood cells. They are seen in thalassemias, enzymopathies, hemoglobinopathies, and after splenectomy.Hookworm Infections: Infection of humans or animals with hookworms other than those caused by the genus Ancylostoma or Necator, for which the specific terms ANCYLOSTOMIASIS and NECATORIASIS are available.Hemoglobinopathies: A group of inherited disorders characterized by structural alterations within the hemoglobin molecule.Cohort Studies: Studies in which subsets of a defined population are identified. These groups may or may not be exposed to factors hypothesized to influence the probability of the occurrence of a particular disease or other outcome. Cohorts are defined populations which, as a whole, are followed in an attempt to determine distinguishing subgroup characteristics.Ifosfamide: Positional isomer of CYCLOPHOSPHAMIDE which is active as an alkylating agent and an immunosuppressive agent.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.Vagus Nerve Stimulation: An adjunctive treatment for PARTIAL EPILEPSY and refractory DEPRESSION that delivers electrical impulses to the brain via the VAGUS NERVE. A battery implanted under the skin supplies the energy.Dogs: The domestic dog, Canis familiaris, comprising about 400 breeds, of the carnivore family CANIDAE. They are worldwide in distribution and live in association with people. (Walker's Mammals of the World, 5th ed, p1065)Methylprednisolone: A PREDNISOLONE derivative with similar anti-inflammatory action.Atrial Fibrillation: Abnormal cardiac rhythm that is characterized by rapid, uncoordinated firing of electrical impulses in the upper chambers of the heart (HEART ATRIA). In such case, blood cannot be effectively pumped into the lower chambers of the heart (HEART VENTRICLES). It is caused by abnormal impulse generation.Dexamethasone: An anti-inflammatory 9-fluoro-glucocorticoid.Cyclosporine: A cyclic undecapeptide from an extract of soil fungi. It is a powerful immunosupressant with a specific action on T-lymphocytes. It is used for the prophylaxis of graft rejection in organ and tissue transplantation. (From Martindale, The Extra Pharmacopoeia, 30th ed).Hemoglobin, Sickle: An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia.Status Epilepticus: A prolonged seizure or seizures repeated frequently enough to prevent recovery between episodes occurring over a period of 20-30 minutes. The most common subtype is generalized tonic-clonic status epilepticus, a potentially fatal condition associated with neuronal injury and respiratory and metabolic dysfunction. Nonconvulsive forms include petit mal status and complex partial status, which may manifest as behavioral disturbances. Simple partial status epilepticus consists of persistent motor, sensory, or autonomic seizures that do not impair cognition (see also EPILEPSIA PARTIALIS CONTINUA). Subclinical status epilepticus generally refers to seizures occurring in an unresponsive or comatose individual in the absence of overt signs of seizure activity. (From N Engl J Med 1998 Apr 2;338(14):970-6; Neurologia 1997 Dec;12 Suppl 6:25-30)Nausea: An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses.Leukemia, Lymphocytic, Chronic, B-Cell: A chronic leukemia characterized by abnormal B-lymphocytes and often generalized lymphadenopathy. In patients presenting predominately with blood and bone marrow involvement it is called chronic lymphocytic leukemia (CLL); in those predominately with enlarged lymph nodes it is called small lymphocytic lymphoma. These terms represent spectrums of the same disease.Malaria, Falciparum: Malaria caused by PLASMODIUM FALCIPARUM. This is the severest form of malaria and is associated with the highest levels of parasites in the blood. This disease is characterized by irregularly recurring febrile paroxysms that in extreme cases occur with acute cerebral, renal, or gastrointestinal manifestations.Intrinsic Factor: A glycoprotein secreted by the cells of the GASTRIC GLANDS that is required for the absorption of VITAMIN B 12 (cyanocobalamin). Deficiency of intrinsic factor leads to VITAMIN B 12 DEFICIENCY and ANEMIA, PERNICIOUS.alpha-Thalassemia: A disorder characterized by reduced synthesis of the alpha chains of hemoglobin. The severity of this condition can vary from mild anemia to death, depending on the number of genes deleted.Topotecan: An antineoplastic agent used to treat ovarian cancer. It works by inhibiting DNA TOPOISOMERASES, TYPE I.Heart Atria: The chambers of the heart, to which the BLOOD returns from the circulation.Electrocardiography: Recording of the moment-to-moment electromotive forces of the HEART as projected onto various sites on the body's surface, delineated as a scalar function of time. The recording is monitored by a tracing on slow moving chart paper or by observing it on a cardioscope, which is a CATHODE RAY TUBE DISPLAY.Spherocytosis, Hereditary: A group of familial congenital hemolytic anemias characterized by numerous abnormally shaped erythrocytes which are generally spheroidal. The erythrocytes have increased osmotic fragility and are abnormally permeable to sodium ions.Antibodies, Monoclonal, Humanized: Antibodies from non-human species whose protein sequences have been modified to make them nearly identical with human antibodies. If the constant region and part of the variable region are replaced, they are called humanized. If only the constant region is modified they are called chimeric. INN names for humanized antibodies end in -zumab.Rh Isoimmunization: The process by which fetal Rh+ erythrocytes enter the circulation of an Rh- mother, causing her to produce IMMUNOGLOBULIN G antibodies, which can cross the placenta and destroy the erythrocytes of Rh+ fetuses. Rh isoimmunization can also be caused by BLOOD TRANSFUSION with mismatched blood.Cisplatin: An inorganic and water-soluble platinum complex. After undergoing hydrolysis, it reacts with DNA to produce both intra and interstrand crosslinks. These crosslinks appear to impair replication and transcription of DNA. The cytotoxicity of cisplatin correlates with cellular arrest in the G2 phase of the cell cycle.Renal Dialysis: Therapy for the insufficient cleansing of the BLOOD by the kidneys based on dialysis and including hemodialysis, PERITONEAL DIALYSIS, and HEMODIAFILTRATION.Mitoxantrone: An anthracenedione-derived antineoplastic agent.Antimicrobial Cationic Peptides: Small cationic peptides that are an important component, in most species, of early innate and induced defenses against invading microbes. In animals they are found on mucosal surfaces, within phagocytic granules, and on the surface of the body. They are also found in insects and plants. Among others, this group includes the DEFENSINS, protegrins, tachyplesins, and thionins. They displace DIVALENT CATIONS from phosphate groups of MEMBRANE LIPIDS leading to disruption of the membrane.Prednisone: A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.Hemoglobinuria, Paroxysmal: A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins.Melphalan: An alkylating nitrogen mustard that is used as an antineoplastic in the form of the levo isomer - MELPHALAN, the racemic mixture - MERPHALAN, and the dextro isomer - MEDPHALAN; toxic to bone marrow, but little vesicant action; potential carcinogen.Hemolysis: The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.Tanzania: A republic in eastern Africa, south of UGANDA and north of MOZAMBIQUE. Its capital is Dar es Salaam. It was formed in 1964 by a merger of the countries of TANGANYIKA and ZANZIBAR.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.Antineoplastic Agents, Phytogenic: Agents obtained from higher plants that have demonstrable cytostatic or antineoplastic activity.Pregnancy Complications, Parasitic: The co-occurrence of pregnancy and parasitic diseases. The parasitic infection may precede or follow FERTILIZATION.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Antisickling Agents: Agents used to prevent or reverse the pathological events leading to sickling of erythrocytes in sickle cell conditions.Parvovirus B19, Human: The type species of ERYTHROVIRUS and the etiological agent of ERYTHEMA INFECTIOSUM, a disease most commonly seen in school-age children.Antigens, CD20: Unglycosylated phosphoproteins expressed only on B-cells. They are regulators of transmembrane Ca2+ conductance and thought to play a role in B-cell activation and proliferation.Folic Acid: A member of the vitamin B family that stimulates the hematopoietic system. It is present in the liver and kidney and is found in mushrooms, spinach, yeast, green leaves, and grasses (POACEAE). Folic acid is used in the treatment and prevention of folate deficiencies and megaloblastic anemia.Pilot Projects: Small-scale tests of methods and procedures to be used on a larger scale if the pilot study demonstrates that these methods and procedures can work.Syndrome: A characteristic symptom complex.

Analysis of the characteristics of folate binding proteins and its relationship with expression of multidrug resistance P-glycoprotein in myelodysplastic syndromes. (1/99)

OBJECTIVE: To observe the characteristics of folate binding proteins (FBP) in myelodysplastic syndromes (MDS) and leukemia and to study the clinical significance of reduced folate carrier (RFC) present in MDS and its relationship with multidrug resistance (MDR). METHODS: The features of FBP on bone marrow cells were analyzed using radiolabeled 3H-folic acid (3H-FA) binding membrane proteins and SDS-polyacrylamide gel electrophoresis (SDS-PAGE). In the same time, P-glucoprotein and mRNA of MDR gene were detected using immunocytochemistry and reverse transcription polymerase chain reaction (RT-PCR) respectively in patients with MDS and leukemia. RESULTS: Two kinds of FBP, folate receptor (FR) and reduced folate carrier (RFC), were found on the leukemic cells. The same results were presented on mononuclear cells of bone marrow in 5 out of 14 MDS patients, and MDR positive was seen in 4 patiens of them. In normal control and other 9 cases of MDS FRs were only found on the mononuclear cells of bone marrow. CONCLUSION: Reduced folate carrier, which is present in the leukemic cell, is a product of neoplastic cell. It might reveal preleukmic state and have the same significance with MDR that RFC is found in MDS patients.  (+info)

Prognostic significance of magnetic resonance imaging of femoral marrow in patients with myelodysplastic syndromes. (2/99)

PURPOSE: To investigate whether the abnormalities observed on femoral marrow magnetic resonance images are related to the development of leukemia and survival of patients with myelodysplastic syndromes (MDS). PATIENTS AND METHODS: The findings on magnetic resonance images of the femoral marrow were evaluated over periods of 1 to 92 months (median, 18 months) in 42 consecutive adult patients with newly diagnosed MDS. Magnetic resonance images were obtained by the T1-weighted spin echo method and the short T1 inversion recovery technique. RESULTS: Magnetic resonance images showed that the femoral marrow patterns changed from fatty, faint, or nodular to scattered or uniform as the disease progressed. Development of acute myeloid leukemia was observed in only 13 patients whose marrow exhibited a scattered or uniform pattern. The overall survival of the 29 patients with a scattered or uniform marrow pattern was significantly shorter than that of the 13 patients with a fatty, faint, or nodular marrow pattern (10.7% v 73.3% at 7 years; P < .01). The period of leukemia-free survival was also significantly shorter in the patients with a scattered or uniform marrow pattern versus a fatty, faint, or nodular pattern (37.7% v 100% at 7 years; P < .01). CONCLUSION: Magnetic resonance images of the femoral marrow can provide valuable information for assessing the prognosis and determining the most appropriate management of patients with MDS.  (+info)

Two cases of myeloid disorders and a t(8;12) (q12;p13). (3/99)

BACKGROUND AND OBJECTIVE: Rearrangements of the short arm of chromosome 12 have been described in different hematologic malignancies. Some of these abnormalities showed a rearrangement of the ETV6 gene. We studied the 12p region in one case with a t(8;12)(q12;p13) by fluorescence in situ hybridization (FISH). DESIGN AND METHODS: We have identified a chromosome translocation, t(8;12)(q12;p13) in two patients with myeloid disorders; one with acute myelogenous leukemia (AML) and one with refractory anemia (RA). FISH studies with specific probes (cosmids and YACs) for the 12p region were used to investigate one case. RESULTS: FISH studies demonstrated hemizygous loss of the ETV6 and CDKN1B regions and two copies of the CCDN2 locus, as a result of the balanced translocation and an additional copy of the der(8). INTERPRETATION AND CONCLUSIONS: Myeloid diseases with t(8;12)(q12;p13) have an interstitial deletion of 12p, including the ETV6 and CDKN1B regions. A duplication of CCDN2 locus can also be found.  (+info)

Prolonged response to cyclosporin-A in hypoplastic refractory anemia and correlation with in vitro studies. (4/99)

BACKGROUND AND OBJECTIVE: Lymphocyte abnormalities in myelodysplastic syndromes (MDS) have been widely described, but the role of the immune system in the pathogenesis of these clonal disorders remains controversial. An active role of lymphocytes in suppressing normal hematopoiesis may be implicated in MDS with hypoplastic marrow. We have studied in vitro and in vivo activity of cyclosporin-A (CSA) on hematopoiesis in patients affected by hypoplastic MDS without blast excess. DESIGN AND METHODS: Nine consecutive patients with hypoplastic refractory anemia (RA), followed up in our out-patient unit, were treated with CSA at daily doses of 1-3 mg/kg for at least three months. Low dose steroids or danazol were transiently added in 7/9 patients. Differences between pre- and post-treatment parameters were studied by the Student's t-test. In vitro effect of CSA on circulating hematopoietic progenitors was studied by the methylcellulose colony assay. RESULTS: Before treatment, fewer circulating hematopoietic progenitors were found in all patients as compared to normal subjects. The number of CD34+ cells was about halved, while circulating erythroid and myeloid colony-forming cells (CFC) were reduced to one-fifth. After a mean period of 22 months of CSA treatment (median: 14.5 months), hemoglobin was significantly and persistently increased in two patients, platelets in one, platelets and hemoglobin in two. Two patients showed transient responses, one patient did not tolerate the treatment and one patient is close to a significant response. At in vitro CSA concentrations similar to those achieved in vivo after oral administration the drug significantly increased cell colony growth in hypoplastic RA. This test correctly predicted a positive clinical response to CSA in 3/5 cases and treatment failure in 4/4 cases. INTERPRETATION AND CONCLUSIONS: About one half of hypoplastic RA patients benefited from CSA treatment. A larger study could verify whether in vitro culture of hematopoietic progenitors in the presence of CSA can predict the clinical response and whether this treatment could prolong patients' survival.  (+info)

Pseudo-Gaucher cells in myelodysplasia. (5/99)

A case of myelodysplastic syndrome is reported, in which the bone marrow contained many cells with the typical light microscopic morphology of Gaucher cells. In the absence of any evidence of inherited Gaucher's disease, these cells are considered to be pseudo-Gaucher cells, which have been described previously in association with other haematological diseases. This is the first report of their occurrence in myelodysplastic syndrome.  (+info)

Successful allogeneic bone marrow transplantation for childhood-onset refractory anemia with ringed sideroblasts. (6/99)

Refractory anemia with ringed sideroblasts (RARS) is an extremely rare type of myelodysplastic syndrome in children. We describe a 10-year-old boy with RARS presented with pancytopenia. He remained relatively stable with only a few transfusions until age of 20 years, when he underwent an allogeneic bone marrow transplantation (BMT) because of increased transfusion requirements. He remains in complete chimeric state at 20 months posttransplant with normal hematologic parameters. To our knowledge, this is the first description of successful BMT in a patient with childhood-onset RARS. The indication of BMT for this rare disorder in children is discussed.  (+info)

Detection of TNFalpha expression in the bone marrow and determination of TNFalpha production of peripheral blood mononuclear cells in myelodysplastic syndrome. (7/99)

TNFalpha is a highly active cytokine which plays an important role in the regulation of apoptotic cell death, a mechanism involved in the pathophysiology of myelodysplastic syndrome (MDS). In this study we investigated the expression of TNFalpha of the bone marrow trephine biopsies by immunohistochemical method and the TNFalpha production of peripheral blood mononuclear cells by ELISA method in 15 patients affected by MDS. Five of seven patients without excess of blasts showed high or intermediate TNFalpha expression in the bone marrow biopsies, whereas two patients with excess of blasts were negative and one had low expression. The five CMML patients revealed low or intermediate expression. The production of TNFalpha by the PBMC was analysed in 10 patients, four patients with RA and two with CMML produced higher level of TNFalpha which increased after stimulation with phorbol myristic acetate, but none of the RAEB patients revealed increase in TNFalpha production. In conclusion we suppose that increased TNFalpha expression and production by PBMC may be an indirect evidence of the role of increased apoptosis in low risk MDS patients.  (+info)

Allelic loss in the progression of myelodysplastic syndrome. (8/99)

To elucidate the genetic events that may play an important role in the progression of myelodysplastic syndrome (MDS) to acute myeloid leukemia (AML), we performed allelotype analysis of 24 individuals using matched MDS and AML samples from the same patients. Because the evolution can take years to occur, we used DNAs extracted from archival samples. These samples were analyzed with 79 microsatellite markers, which mapped to each of the autosomal arms except the short arms of the acrocentric chromosomes. Loss of heterozygosity on at least one locus was observed in 18 of the 24 cases (75%) as the disease progressed. Frequent allelic loss in >20% of the informative cases was observed on chromosome arms 6q (31%), 7p (23%), 10p (31%), 11q (27%), 14q (25%), and 20q (23%). Although cytogenetic information was available for many of our cases with allelic loss on 6q, 7p, 10p, 11q, 14q, and 20q, no deletions were observed on these arms. Fractional allelic loss, calculated for each sample as the total number of chromosomal arms lost per total number of arms with information, showed a median value of 0.06 and a mean of 0.15 (range, 0-0.59). No microsatellite instability at more than one marker was found in any of the samples. These results suggest that tumor suppressor genes exist on 6q, 7p, 10p, 11q, 14q, and 20q that have an important role in the evolution of MDS to AML when they are mutated.  (+info)

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MGI Pharma, Inc, and SuperGen, Inc, recently announced that the US Food and Drug Administration (FDA) has approved the hypomethylating agent decitabine (Dacogen) for injection. Decitabine is indicated for treatment of patients with myelodysplastic syndromes (MDS) including previously treated and untreated, de novo, and secondary MDS of all French-American-British (FAB) subtypes (refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and chronic myelomonocytic leukemia), and intermediate-1, intermediate-2, and high-risk International Prognostic Scoring System (IPSS) groups. 1
Learn in-depth information on Refractory Anemia with Excess Blasts, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis.
in Blood (1998), 92(1), 68-75. Treatment with erythropoietin (epo) may improve the anemia of myelodysplastic syndromes (MDS) in approximately 20% of patients. Previous studies have suggested that treatment with the combination of ... [more ▼]. Treatment with erythropoietin (epo) may improve the anemia of myelodysplastic syndromes (MDS) in approximately 20% of patients. Previous studies have suggested that treatment with the combination of granulocyte colony-stimulating factor (G-CSF) and epo may increase this response rate. In the present phase II study, patients with MDS and anemia were randomized to treatment with G-CSF + epo according to one of two alternatives; arm A starting with G-CSF for 4 weeks followed by the combination for 12 weeks, and arm B starting with epo for 8 weeks followed by the combination for 10 weeks. Fifty evaluable patients (10 refractory anemia [RA], 13 refractory anemia with ring sideroblasts [RARS], and 27 refractory anemia with excess blasts [RAEB]) were included ...
OUTLINE: This is a multicenter study. Patients are stratified according to myelodysplastic syndrome subclassification (refractory anemia [RA] vs RA with ringed sideroblasts vs RA with excess blasts).. Patients receive induction therapy comprising anti-thymocyte globulin IV over 6-12 hours on days 1-4 and oral cyclosporine twice daily on days 5-94 followed by a taper until day 124. Patients who relapse after a response of at least 60 days may receive reinduction therapy comprising oral cyclosporine twice daily on days 1-90 followed by a taper until day 120. Treatment continues in the absence of disease progression or unacceptable toxicity.. Patients are followed monthly for 6 months, every 2 months for 2 years, and then every 6 months for 3 years.. PROJECTED ACCRUAL: A total of 130 patients (53 with refractory anemia [RA], 33 with RA with ringed sideroblasts, and 44 with RA with excess blasts) will be accrued for this study within 14-22 months. ...
OUTLINE: This is a multicenter study. Patients are stratified according to myelodysplastic syndrome subclassification (refractory anemia [RA] vs RA with ringed sideroblasts vs RA with excess blasts).. Patients receive induction therapy comprising anti-thymocyte globulin IV over 6-12 hours on days 1-4 and oral cyclosporine twice daily on days 5-94 followed by a taper until day 124. Patients who relapse after a response of at least 60 days may receive reinduction therapy comprising oral cyclosporine twice daily on days 1-90 followed by a taper until day 120. Treatment continues in the absence of disease progression or unacceptable toxicity.. Patients are followed monthly for 6 months, every 2 months for 2 years, and then every 6 months for 3 years.. PROJECTED ACCRUAL: A total of 130 patients (53 with refractory anemia [RA], 33 with RA with ringed sideroblasts, and 44 with RA with excess blasts) will be accrued for this study within 14-22 months. ...
Refractory Anaemia (RA) is part of the heterogeneous group of diseases that affects normal blood cell production in the bone marrow and a category of myelodysplastic syndrome (MDS) . One example of RA is the 5q-syndrome. In RA, marrow blood cells fail to mature properly and are unable to work properly. They often die before they leave the marrow, or shortly after reaching the bloodstream (ineffective erythropoiesis or dyserythropoiesis). There are few data on the epidemiology of RA, which may account for 30-40% of all MDS cases. MDS is predominantly diagnosed in the elderly population. The global incidence of all MDS was comprised between 3,5 and 12,6 new cases / year / per 100,000 in some studies. ...
BACKGROUND: Myelodysplastic syndromes are a diverse and common group of chronic hematologic cancers. The identification of new genetic lesions could facilitate new diagnostic and therapeutic strategies. METHODS: We used massively parallel sequencing technology to identify somatically acquired point mutations across all protein-coding exons in the genome in 9 patients with low-grade myelodysplasia. Targeted resequencing of the gene encoding RNA splicing factor 3B, subunit 1 (SF3B1), was also performed in a cohort of 2087 patients with myeloid or other cancers. RESULTS: We identified 64 point mutations in the 9 patients. Recurrent somatically acquired mutations were identified in SF3B1. Follow-up revealed SF3B1 mutations in 72 of 354 patients (20%) with myelodysplastic syndromes, with particularly high frequency among patients whose disease was characterized by ring sideroblasts (53 of 82 [65%]). The gene was also mutated in 1 to 5% of patients with a variety of other tumor types. The observed mutations
Immunohistochemistry has become a very important, and in some cases indispensable, tool in diagnostic pathology, enabling the precise identification of tumours, the detection of micrometastases in a given sample, and the evaluation of various prognosis factors. However, in some cases, the use of multiple but distinct immunostains can lead to some unforeseen results-for example, the expression of an apparently aberrant marker by a neoplasm can sometimes be seen. In this context, we report our experience with a case of refractory anaemia with excess of blasts in transformation (RAEB-t) in which the blasts were unexpectedly found to express cytokeratin (CK).. An 86 year old woman with a past medical history of breast carcinoma treated by mastectomy and adjuvant radiotherapy was admitted to our institution because of worsening anaemia. The following haematological indices were noticed: haemoglobin, 8.6 g/litre; erythrocytes, 2.5 × 1012/litre; white blood cells, 3 × 109/litre; and platelets, 465 × ...
Question - Refractory anaemia,very low counts,works as a builder,has pacemaker,ulcerated leg after fall,treatment and prognosis?. Ask a Doctor about Blood transfusion, Ask a General & Family Physician
Because of the diversity of clinical symptoms, the diagnosis of mitochondrial DNA (mtDNA) deletion disorders can be difficult. Here, we describe an 8-month-old boy presenting clinically exclusively with refractory anemia. Mutation analysis in our pat
In refractory anemia (RA) and refractory anemia with ringed sideroblasts (RARS) a discrepancy is observed between the decreased in vitro erythroid colony formation and the normal or increased number of normoblasts in the bone marrow. To study the in
Acute erythroid leukemia or Di Guglielmo syndrome is a rare form of acute myeloid leukemia (less than 5% of AML cases) where the myeloproliferation is of erythroblastic precursors. It is defined as type "M6" under the FAB classification. The most common symptoms of AEL are related to pancytopenia (a shortage of all types of blood cells), including fatigue, infections, and mucocutaneous bleeding. Almost half of people with AEL exhibit weight loss, fever and night sweats at the time of diagnosis. Almost all people with AEL are anemic, and 77% have a hemoglobin level under 10.0 g/dl. Signs of thrombocytopenia are found in about half of people with AEL. The causes of AEL are unknown. Prior to a 2008 reclassification by the World Health Organization, cases that evolved from myelodysplastic syndromes, myeloproliferative neoplasms, chemotherapy for other cancers or exposure to toxins were defined as secondary AEL. These cases are now likely to instead be classified as acute myeloid leukemia with ...
The British Society for Haematology is registered in England and Wales as a Company Limited by Guarantee, No 2645706 and as a Charity, No 1005735 Registered Office and correspondence address: 100 White Lion Street London N1 9PF. Phone: 020 7713 0990 ...
My 11 week old son has just been diagnosed with acute erythroid leukemia, he has just received a liver transplant last week (due to inability to perform biopsy pre transplant), he will receive chemo when he recovers from transplant. Seems pretty rare, anyone know of any successful cases around as most seem to be unfortunately post mortem?. Reply Follow This Thread Stop Following This Thread Flag this Discussion ...
SF3B1 to be frequently mutated in cases of MDS with ring sideroblasts, specifically refractory anemia with ring ... linking SF3B1 dysfunction and the formation of ring sideroblast in MDS. He is also very active in conducting clinical trials .... Grant Recipient last updated 10/30/2012 - 6:28pm.. ...
The British Society for Haematology is registered in England and Wales as a Company Limited by Guarantee, No 2645706 and as a Charity, No 1005735 Registered Office and correspondence address: 100 White Lion Street London N1 9PF. Phone: 020 7713 0990 ...
Free, official coding info for 2020 ICD-10-CM D46.20 - includes detailed rules, notes, synonyms, ICD-9-CM conversion, index and annotation crosswalks, DRG grouping and more.
Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day ...
PLOS ONE 2010;5(12):e14398-. Gene expression profiling of day 7 erythroblasts from refractory anemia with ringed sideroblasts (RARS) and microarray-based identification of erythroid granulocyte-CSF (G-CSF) targets ...
Patients. We searched the AML database for patients who presented to The University of Texas M. D. Anderson Cancer Center with newly diagnosed AML (≥20% myeloblasts) from 1990 through 2005. This database includes consecutive patients with AML or MDS seen at M. D. Anderson in the Department of Leukemia since 1985. Patients previously classified with refractory anemia with excess blasts in transformation were reclassified as AML. A total of 2,014 patients were identified, and pretreatment levels of β2M were available in 64% (i.e., 1,293 patients). Serum β2M levels were quantified by RIA (Pharmacia β-2 Micro Ria; Pharmacia Diagnostic; reference range, 0.7-2.0 mg/L). Treatment for AML varied during the 16 years depicted here, and for convenience we divided the patients into those who were given 1-β-d-arabinofuranosylcytosine (ara-C) and those who were not. All patients included in the prognostic models received remission induction therapy with high-dose ara-C, defined as ,0.5 g daily for 3 to ...
Looking for online definition of refractory anemia with excess blasts in the Medical Dictionary? refractory anemia with excess blasts explanation free. What is refractory anemia with excess blasts? Meaning of refractory anemia with excess blasts medical term. What does refractory anemia with excess blasts mean?
The myelodysplastic syndromes (MDS) are a group of clonal hematopoietic disorders characterized by bone marrow failure and a risk of progression to acute myeloid leukemia (AML). Anemia affects the course of disease, quality of life (QOL), and cognitive function of MDS patients. Erythroid-stimulating agents (ESAs) are effective; however, not all patients respond to ESAs. To evaluate the effectiveness of a biosimilar epoetin α (Binocrit) for the treatment of anemia in low-/intermediate-1 risk MDS patients and to evaluate the impact of ESAs on QOL and on
... Primary Hereditary Sideroblastic Anemia. Primary Acquired Refractory Anemia With Ringed Sideroblasts (RARS). Sideroblastic Anemia Information Including: BASIC INFORMATION, SIGNS AND SYMPTOMS, EPIDEMIOLOGY & DEMOGRAPHICS, PHYSICAL FINDINGS & CLINICAL PRESENTATION, LABORATORY TESTS. DIAGNOSIS, TREATMENT and more
Azacitidine has proved prolonged overall survival in patients with high-risk MDS. Minor pilot studies have shown that treatment with Azacitidine can ind
OBJECTIVE: The current therapy of myelodysplastic syndrome (MDS) is unsatisfactory and comprises mainly supportive treatment or antileukemic chemotherapy. Recent studies about successful immunosuppressive therapy suggest an autoimmune mechanism in subtypes of myelodysplastic syndrome. PATIENTS AND METHODS: To investigate this hypothesis, bone marrow mononuclear cells (MNC) from 15 patients with low-grade MDS, refractory anemia, and refractory anemia with ringed sideroblasts (RA and RARS), and from 7 normal donors were depleted of CD2(+), CD5(+), and CD7(+) lymphocytes using magnetic cell sorting. Depleted and nondepleted MNC were seeded onto irradiated allogeneic bone marrow stroma and the generation of colony-forming-cells (CFC), the clonal origin of hemopoietic progenitor cells in long-term bone marrow culture (LTC), was compared. RESULTS: The capacity of MNC from 7 healthy donors to generate hemopoiesis remained unchanged in the lymphocyte-depleted LTC. In contrast, cultures initiated with ...
BACKGROUND AND OBJECTIVE: Anemia leading to transfusion is probably the most important problem in patients with myelodysplastic syndromes (MDS). Human recombinant erythropoietin (rHuEpo) and granulocyte colony-stimulating factor (G-CSF) have been used to treat patients with anemia of MDS, but fewer than 50% respond. The aim of this work was to evaluate the benefit of rHuEpo +/- G-CSF treatment and to isolate the response predictive variables in a group of selected patients with MDS. DESIGN AND METHODS: A non-randomized multicenter trial was carried out in 32 patients with MDS. The inclusion criteria were age ,= 18 years, refractory anemia (RA) or refractory anemia with ringed sideroblasts, Hb ,= 100 g/L or receiving transfusions and serum erythropoietin ,= 250 U/L. These patients were treated with subcutaneous rHuEpo (300 U/kg) three times a week for 8 weeks. In the case of partial response (PR) or no response (NR) subcutaneosly administered G-CSF (1 microg/kg) three times a week was added to ...
In a previous study, we identified somatic mutations of SF3B1, a gene encoding a core component of RNA splicing machinery, in patients with myelodysplastic syndrome (MDS). Here, we define the clinical significance of these mutations in MDS and myelodysplastic/myeloproliferative neoplasms (MDS/MPN). The coding exons of SF3B1 were screened using massively parallel pyrosequencing in patients with MDS, MDS/MPN, or acute myeloid leukemia (AML) evolving from MDS. Somatic mutations of SF3B1 were found in 150 of 533 (28.1%) patients with MDS, 16 of 83 (19.3%) with MDS/MPN, and 2 of 38 (5.3%) with AML. There was a significant association of SF3B1 mutations with the presence of ring sideroblasts (P , .001) and of mutant allele burden with their proportion (P = .002). The mutant gene had a positive predictive value for ring sideroblasts of 97.7% (95% confidence interval, 93.5%-99.5%). In multivariate analysis including established risk factors, SF3B1 mutations were found to be independently associated with ...
It has been suggested that myeloid neoplasms with isolated isochromosome 17q[MN i(17q)] comprise a distinct entity with poor prognosis. However, literature reports show a considerable clinical and molecular heterogeneity. We describe a 58-year-old male patient who was diagnosed as refractory anemia with multilineage dysplasia and ringed sideroblasts with isolated i(17q). Though he initially responded well to erythropoietin, he gradually progressed to an aggressive form of MDS/MPN refractory to azacytidine and died 29 months after first diagnosis. Notably, in contrast to disease advancement, his karyotype reverted to normal, whereas his mutational profile remained unchanged. To our knowledge this is the first report of karyotype normalization during disease progression in patients with MN i(17q), suggesting that the i(17q) anomaly is dispensable for the leukemic transformation and highlighting the underlying clinical and molecular complexity which both have to be resolved before the establishment ...
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Objective: Anaemia in low-risk myelodysplastic syndromes (MDS) is associated with reduced quality of life (QoL). Response to treatment with erythropoietin +/- granulocyte colony-stimulating factor (G-CSF) is associated with improved QoL, but whether transfusion therapy with higher haemoglobin (Hb) target levels has similar effects is unknown. The objective for this prospective phase II Nordic multicentre trial was to assess QoL, response rate and physical function in elderly anaemic MDS patients treated to a target Hb level of andgt; 120 g /L. Methods: Thirty-six elderly patients with low-and intermediate-1 risk MDS received darbepoetin (DA) 300 mu g/wk, with the addition of G-CSF if no response. If the Hb target was reached at 16 wk, treatment was maintained until week 26. Remaining patients were transfused to reach the target level for at least 8 wk. Results: Twenty-seven patients completed the study. Response rate to DA +/- G-CSF was 67% in evaluable patients and 56% according to intention to ...
A 60-year-old woman presented with weakness and a history of multiple blood transfusions in the past 6 months. A complete blood count showed the following: hemoglobin concentration, 7.8 g/dL; platelet count, 30×109/L; and leucocyte count, 23.1×109/L. The peripheral blood smear showed the presence of dysplastic eosinophils (50%) with abnormally large purple-black basophilic granules, hypogranular neutrophils with pseudo-Pelger-Hüet anomaly (A, arrow; May-Grünwald-Giemsa stain, ×1,000), and 2% blasts. The bone marrow was hypercellular and showed features of refractory anemia with excess blasts (RAEB-2) with 15% blasts, dysplastic megakaryocytes (B, arrow), and numerous eosinophilic precursors with abnormally large granules (C) with strong myeloperoxidase positivity, hence confirming that these were dysplastic eosinophils (D). Conventional cytogenetics revealed major karyotype abnormalities (MAKA) with monosomy in chromosomes 5, 7, 8, 20, and 21, deletion in the short arm of 11, t(2;6), and ...
With increasing recognition of newly identified molecular features, the 2016 revision to the WHO classification includes several changes in the myeloid and lymphoid categories, with improved characterization of diseases. Growing data has become available on recurring mutations and pattern of co-mutations in many hematopoietic neoplasms that has allowed recognition of new entities and refined the diagnostic criteria of existing entities. Among myeloid neoplasms, newly discovered molecular genetic abnormalities have resulted in a new myeloid/lymphoid neoplasm disease entity associated with PCM1-JAK2 rearrangement and have enhanced our understanding of myelodysplastic syndromes with ring sideroblasts. Recognition of myeloid neoplasms occurring in the background of germline predisposing mutations is also emphasized in the revision and a new disease category has been created to encompass this group. Among the lymphomas, there has been clarification of certain lymphoma subtypes that most commonly ...
E-Cadherin is calcium-dependent cell adhesion molecule encoded by CDH1 gene at chromosome 16q22.1. Ecadherin regulates cell-cell adhesions and controls mobility and proli..
Mutations of spliceosome components are common in myeloid neoplasms. One of the affected genes, PRPF8, encodes the most evolutionarily conserved spliceosomal protein. We identified either recurrent somatic PRPF8 mutations or hemizygous deletions in 15/447 and 24/450 cases, respectively. Fifty percent of PRPF8 mutant and del(17p) cases were found in AML and conveyed poor prognosis. PRPF8 defects correlated with increased myeloblasts and ring sideroblasts in cases without SF3B1 mutations. Knockdown of PRPF8 in K562 and CD34+ primary bone marrow cells increased proliferative capacity. Whole-RNA deep sequencing of primary cells from patients with PRPF8 abnormalities demonstrated consistent missplicing defects. In yeast models, homologous mutations introduced into Prp8 abrogated a block experimentally produced in the second step of the RNA splicing process, suggesting that the mutants have defects in proof-reading functions. In sum, the exploration of clinical and functional consequences suggests that PRPF8
What is myelodysplastic syndromes mds? Learn about myelodysplastic syndromes mds symptoms, myelodysplastic syndromes mds causes, diagnosis, and more.
What is myelodysplastic syndromes mds? Learn about myelodysplastic syndromes mds symptoms, myelodysplastic syndromes mds causes, diagnosis, and more.
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FYI - According to a recent article in the Journal of Clinical Oncology re: myelodysplastic syndromes (MDS): April 30, 2010 - Myelodysplastic syndromes (MDS) appear to be nearly 5 times more common...
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Learn more about Managing the Side Effects of Myelodysplastic Syndrome (MDS) and MDS Treatment at Doctors Hospital of Augusta Main Page ...
The myelodysplastic syndromes (MDS) encompass a series of hematologic conditions characterized by chronic cytopenias (anemia, neutropenia, thrombocytopenia) accompanied by abnormal cellular maturation. As a result, patients with MDS are at risk for s
Learn more about Diagnosis and Prognosis of Myelodysplastic Syndrome (MDS) at Doctors Hospital of Augusta Main Page Risk Factors ...
Learn more about Cancer In Depth: Myelodysplastic Syndrome (MDS) at Coliseum Health System Main Page Risk Factors ...
Learn more about Other Treatments for Myelodysplastic Syndrome (MDS) at Grand Strand Medical Center Main Page Risk Factors ...
For more videos by Vernon Louw MedEd, go to: http://www.youtube.com/user/Legomed/videos?view_as=public This video explains the natural course of disease in patients with MDS and the principles ...
Characteristic features of myelodysplastic syndrome (MDS) in humans. MDS is thought to originate from a mutated Hematopoietic stem cell (HSC). Approximately 30%
The long-term goal of this project is to identify and characterize genes that contribute to the development and progression of myelodysplastic syndromes (MDS)....
For more videos by Vernon Louw MedEd, go to: http://www.youtube.com/user/Legomed/videos?view_as=public This video explains the natural course of disease in patients with MDS and the principles ...
The purpose of this study is to determine the overall response rate in patients with myelodysplastic syndromes (MDS) given a daily dosing schedule of de
"Severe Anemia". Hart GB, Lennon PA, Strauss MB (1987). "Hyperbaric oxygen in exceptional acute blood-loss anemia". J. ... "Refractory Osteomyelitis". Retrieved 2011-08-21. Mader JT, Adams KR, Sutton TE (1987). "Infectious diseases: pathophysiology ... refractory); Delayed radiation injury (soft tissue and bony necrosis); Skin grafts and flaps (compromised); Thermal burns. ... anemia); Idiopathic sudden sensorineural hearing loss; Intracranial abscess; Mucormycosis, especially rhinocerebral disease in ...
GLRX5 Anemia, sideroblastic, pyridoxine-refractory, autosomal recessive; 205950; SLC25A38 Anemia, sideroblastic, with ataxia; ... RHAG Anemia, hypochromic microcytic; 206100; NRAMP2 Anemia, sideroblastic, pyridoxine-refractory, autosomal recessive; 205950; ... SCN5A Heinz body anemia; 140700; HBA2 Heinz body anemias, alpha-; 140700; HBA1 Heinz body anemias, beta-; 140700; HBB HELLP ... RPL5 Diamond-Blackfan anemia 7; 612562; RPL11 Diamond-Blackfan anemia 8; 612563; RPS7 Diamond-Blackfan anemia 9; 613308; RPS10 ...
Mutations in this gene are associated with autosomal recessive pyridoxine-refractory sideroblastic anemia. The GLRX5 gene ... as well as pyridoxine-refractory, autosomal recessive anemia (PRARSA). Cells with mutations in GLRX5 activity show deficiency ... Mutations in the GLRX5 gene have been associated with sideroblastic anemia, variant glycine encephalopathy (also known as non- ... Camaschella C (Oct 2008). "Recent advances in the understanding of inherited sideroblastic anaemia". British Journal of ...
Huguley CM, Bain JA, Rivers SL, Scoggins RB (Jun 1959). "Refractory megaloblastic anemia associated with excretion of orotic ... It causes a characteristic form of anemia and may be associated with mental and physical retardation. Orotic acid is an ... In addition to the characteristic excessive orotic acid in the urine, patients typically have megaloblastic anemia (UMP ... Orotic aciduria is associated with megaloblastic anemia due to decreased pyrimidine synthesis, which leads to decreased ...
It is associated with diaphyseal dysplasia and refractory anemia. It is associated with a deficiency of Thromboxane-A synthase ... Ghosal SP, Mukherjee AK, Mukherjee D, Ghosh AK (July 1988). "Diaphyseal dysplasia associated with anemia". J. Pediatr. 113 (1 ...
15 May 2003). "Rituximab for the treatment of refractory autoimmune hemolytic anemia in children". Blood. 101 (10): 3857-61. ... Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen and carbon dioxide are ... Liu H, Shao Z, Jing L (2001). "[The effectiveness of cyclosporin A in the treatment of autoimmune hemolytic anemia and Evans ... The diagnosis is made upon blood tests to confirm not only hemolytic anemia and immune thrombocytopenic purpura, but also a ...
... mutations are found in 60%-80% of patients with refractory anemia with ring sideroblasts (RARS; which is a ...
"A new syndrome of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreatic dysfunction ... The syndrome also leads to anemia, low platelet count, and aplastic anemia It may be confused with transient erythroblastopenia ... Pearson Syndrome is a very rare mitochondrial disorder that is characterized by health conditions such as sideroblastic anemia ... The symptoms of Pearson Syndrome are mitochondrial cytopathy with anemia, neutropenia, and thrombocytopenia. Pearson, Howard A ...
"Eltrombopag restores trilineage hematopoiesis in refractory severe aplastic anemia that can be sustained on discontinuation of ... In 2017, the NIH made Eltrombopag a standard of care in aplastic anemia. It has been shown to produce a trilineage hematopoesis ... Eltrombopag received FDA breakthrough treatment designation in February 2014 for patients with aplastic anemia for which ... "Eltrombopag Added to Standard Immunosuppression for Aplastic Anemia". New England Journal of Medicine. 376 (16): 1540-1550. doi ...
Anemia[edit]. Megaloblastic anemia (MA) is associated with GSE and is believed to be the result of B12 and folate deficiency.[ ... This condition is known as refractory celiac disease (RCD), defined as malabsorption due to gluten-related enteropathy (villous ... Iron-deficiency anemia. Iron-deficiency anemia (IDA) may be the only symptom for CD,[26] detected in subclinical CD[27] and is ... Pernicious anemia (PA). Pernicious anemia is associated with GSE and is believed to result primarily from malabsorption ...
2003). "Inappropriate expression of hepcidin is associated with iron refractory anemia: implications for the anemia of chronic ... 2003). "Hepcidin, a putative mediator of anemia of inflammation, is a type II acute-phase protein". Blood. 101 (7): 2461-3. doi ... Roy CN, Andrews NC (2005). "Anemia of inflammation: the hepcidin link". Curr. Opin. Hematol. 12 (2): 107-11. doi:10.1097/ ... a key regulator of iron metabolism and mediator of anemia of inflammation". Blood. 102 (3): 783-8. doi:10.1182/blood-2003-03- ...
... inTreating Older Patients With Newly Diagnosed Acute Myeloid Leukemia or Refractory Anemia - Full Text View - ...
... an increased risk of potentially fatal aplastic anemia and/or liver failure limit the drug's usage to severe refractory ... Two rare but very serious effects include aplastic anemia and hepatic (liver) failure. The risk of aplastic anemia is between 1 ... It was urgently withdrawn after 10 cases of aplastic anemia. A "Dear Doctor" letter was sent to 240,000 physicians. September ...
... can be aberrantly expressed in refractory anaemia with excess blasts (RAEB) and may confer a worse prognosis. Also, a lack ...
"Effect of subcutaneous deferoxamine and oral vitamin C on iron excretion in congenital hypoplastic anemia and refractory anemia ... with β-thalassemia trait should be warned that their condition can be misdiagnosed for the common Iron deficiency anemia. They ...
... may be caused by vitamin deficiency or chemotherapy, or it may be a sign of refractory anemia, which is a ...
"Megaloblastic Anemia in young children". PubMed. "Pediatrics and Therapeutics: Current Research". BioInfo. ... "Efficacy of ketogenic ratio diets in refractory epilepsy in young children". PubMed. "Indira Gandhi Institute of Child Health ... critical conditions such as Myoclonic Seizure and Megaloblastic anemia. He is also the editorial board member of Pediatrics and ...
They are also used to distinguish the pre-leukemia myelodysplastic syndromes: refractory anemia with excess blasts 2 (which has ...
Failure to thrive and anaemia may also be present. Lower gastrointestinal bleeding may imply colonic involvement. Muscular EG ( ... Other documented features are cholangitis, pancreatitis, eosinophilic splenitis, acute appendicitis and giant refractory ...
Iron-deficiency anemia. Iron-deficiency anemia (IDA) may be the only symptom for CD, detected in subclinical CD and is ... In one instance rhuematoid arthritis was tied directly to refractory disease. Still's disease (AOSD) is a rheumatic disorder of ... B12 deficiency Megaloblastic anemia Pernicious anemia Vitamin D deficiency. Vitamin D deficiency can result in osteopenia and ... Pernicious anemia (PA). Pernicious anemia is associated with GSE and is believed to result primarily from malabsorption ...
... positive cells from bone marrow of patients with myelodysplastic syndromes with the highest expression in refractory anemia ...
Development of refractory delusional parasitosis is possible, as a person develops an overwhelming obsession with bed bugs. A ... Due to each bite taking a tiny amount of blood, chronic or severe infestation may lead to anemia. Bacterial skin infection may ...
"Refractory" CLL is a disease that no longer responds favorably to treatment. In this case, more aggressive therapies, including ... In some individuals, the disease comes to light only after the cancerous cells overwhelm the bone marrow resulting in anemia ... Enlargement of the spleen and anemia may also occur. It typically worsens gradually. Risk factors include having a family ... The monoclonal antibody alemtuzumab (directed against CD52) may be used in patients with refractory, bone marrow-based disease ...
If the individual doesn't respond to steps 1-3 then they are considered to have refractory symptoms. At this point, anti- ... Sulfasalazine is a good option for people with anemia who cannot take dapsone. Hydroxychloroquine is an antimalarial agent that ... Other options for refractory symptoms of chronic hives include anti-inflammatory medications, omalizumab, and ... It is helpful in therapy-refractory cases and is contraindicated in patients with G6PD deficiency. Sulfasalazine, a 5-ASA ...
... and an anemia which in many patients is profound, refractory to traditional therapies (e.g. iron supplements, Erythropoietin), ... They present with anemia, increases in blood eosinophils and monocytes, splenomegaly, and, less often, lymphadenopathy. Bone ... They commonly present with anemia, eosinophilia, monocytosis, and splenomegaly and have their disease classified as chronic ...
Other autoimmune diseases that have been treated with rituximab include autoimmune hemolytic anemia, pure red cell aplasia, ... April 2005). "Rituximab chimeric anti-CD20 monoclonal antibody treatment for adult refractory idiopathic thrombocytopenic ... chronic inflammatory demyelinating polyneuropathy and autoimmune anemias.[16] The most dangerous, although among the most rare ... be an effective rheumatoid arthritis treatment in three randomised controlled trials and is now licensed for use in refractory ...
Treatment of refractory autoimmune haemolytic anaemia with anti-CD20 (Rituximab). Br J Haematol2001;114:241-6. ... We report the case of an 8 week old infant with fulminant autoimmune haemolytic anaemia refractory to conventional ... Paediatricians should be aware of this useful therapeutic tool for treatment of refractory autoimmune haemolytic anaemia and ... Rituximab: a very efficient therapy in cold agglutinins and refractory autoimmune haemolytic anaemia associated with CD-20 ...
To the best of our knowledge, this is the first reported case of mixed-type autoimmune hemolytic anemia that did not respond to ... We report a case of severe mixed autoimmune hemolytic anemia that did not respond to steroids and responded to four weekly ... Rituximab was continued for a total of four weeks and led to the complete resolution of his hemolytic anemia and associated ... His blood work revealed severe anemia (hemoglobin, 4.9 g/dL) with biochemical evidence of hemolysis. Exposure to cold led to ...
A 28 year old female patient presented with refractory anaemia since childhood and recurrent still-births at 28-30 weeks of ... N2 - A 28 year old female patient presented with refractory anaemia since childhood and recurrent still-births at 28-30 weeks ... AB - A 28 year old female patient presented with refractory anaemia since childhood and recurrent still-births at 28-30 weeks ... abstract = "A 28 year old female patient presented with refractory anaemia since childhood and recurrent still-births at 28-30 ...
Primary Hereditary Sideroblastic Anemia. Primary Acquired Refractory Anemia With Ringed Sideroblasts (RARS). Sideroblastic ... Basic Information, Causes, Diagnosis and Treatment of Sideroblastic Anemia. ... Anemia Information Including: BASIC INFORMATION, SIGNS AND SYMPTOMS, EPIDEMIOLOGY & DEMOGRAPHICS, PHYSICAL FINDINGS & CLINICAL ... Primary hereditary sideroblastic anemia. • Primary acquired refractory anemia with ringed sideroblasts (RARS). • Secondary ...
Iron-refractory iron deficiency anemia is one of many types of anemia, which is a group of conditions characterized by a ... medlineplus.gov/genetics/condition/iron-refractory-iron-deficiency-anemia/ Iron-refractory iron deficiency anemia. ... Iron-refractory iron deficiency anemia is one of many types of anemia. , which is a group of conditions characterized by a ... Although iron deficiency anemia is relatively common, the prevalence of the iron-refractory form of the disease is unknown. At ...
Vorinostat in Treating Patients With Relapsed or Refractory Acute Leukemia or Chronic Myelogenous Leukemia or Refractory Anemia ... Zosuquidar inTreating Older Patients With Newly Diagnosed Acute Myeloid Leukemia or Refractory Anemia. *Leukemia ... Identification of Fanconi anemia patients. 20. All. up to 21 Years (Child, Adult). NCT01146210. AAML10B2. NCI-2011-02219. COG- ... Identification of de Novo Fanconi Anemia in Younger Patients With Newly Diagnosed Acute Myeloid Leukemia. *Childhood Acute ...
Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA).. Finberg KE1, Heeney MM, Campagna DR, Aydinok Y, ... Here, we show that iron deficiency anemia refractory to oral iron therapy can be caused by germline mutations in TMPRSS6, which ... Iron Deficiency Anemia - Genetic Alliance. *Anemia - Genetic Alliance. *Iron-refractory iron deficiency anemia - Genetics Home ...
We describe the cases of three children with chronic active Helicobacter pylorigastritis and iron-deficiency anaemia without ... Refractory iron-deficiency anaemia due to silent Helicobacter pylori gastritis in children. ... Barabino A, Dufour C, Marino CM, Claudiani F, Alessandri A (1999) Unexplained refractory iron-deficiency anemia associated with ... Helicobacter pylori gastric infection and sideropenic refractory anemia. J Pediatr Gastroenterol Nutr 17:225-227CrossRefPubMed ...
Refractory anemia with ring sideroblasts (RARS) is a type of myelodysplastic syndrome. RARS is characterized by 5% or less ... RARS is distinguished from refractory anemia by having 15% or more ringed sideroblasts among the erythroid precursors in the ... "Pathology of Refractory Anemia With Ring Sideroblasts". Retrieved 28 August 2013. ...
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... refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, or acute myeloid leukemia [AML]) ... Refractory anemia with excess of blasts (RAEB) is a type of myelodysplastic syndrome with a marrow blast percentage of 5% to 19 ... July 2006). "An antecedent diagnosis of refractory anemia with excess blasts has no prognostic relevance in acute myeloid ...
Alefacept in Patients With Relapsed/Refractory Aplastic Anemia. The safety and scientific validity of this study is the ... A Phase 1/2 Study of Alefacept, a CD2 Receptor Antagonist in Patients With Relapsed/Refractory Aplastic Anemia. ... To define the safety, tolerability, dose-limiting toxicities (DLT), of alefacept in relapsed/ refractory aplastic anemia (AA). ... Aplastic Anemia (AA) is an autoimmune hematologic stem cell disease mediated by activated T-lymphocytes that leads to ...
iron deficiency anemia. IRIDA - iron-refractory iron deficiency anemia. MCV - mean corpuscular volume. WPPSI-IV - Wechsler ... Iron-Refractory Iron Deficiency Anemia May Not Lead to Neurocognitive Dysfunction: A Case Report. Valérie Arsenault, Chantal ... Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA). Nat Genet. 2008;40(5):569-571pmid:18408718. ... A novel mutation Gly603Arg of TMPRSS6 in a Korean female with iron-refractory iron deficiency anemia. Pediatr Blood Cancer. ...
NICE is unable to make a recommendation about the use in the NHS of Eltrombopag for treating severe aplastic anaemia refractory ... Eltrombopag for treating severe aplastic anaemia refractory to immunosuppressive therapy (terminated appraisal). Technology ... NICE was unable to make recommendations on eltrombopag (Revolade) for severe aplastic anaemia refractory to immunosuppressive ...
... refractory anaemia explanation. Define refractory anaemia by Websters Dictionary, WordNet Lexical Database, Dictionary of ... anaemia, anemia, refractory anemia, sideroblastic anaemia, sideroblastic anemia, siderochrestic anaemia, siderochrestic anemia ... Refractory. -- refractory anaemia --. refractory anemia. refractory period. refractory pot. refractory-lined. Refracture. ... refractory anaemia Noun. 1.. refractory anaemia - any of various anemic conditions that are not successfully treated by any ...
... in participants with relapsed/refractory acute myeloid leukemia (AML) who have exhausted standard available therapies known to ... Study of PTC299 in Relapsed/Refractory Acute Leukemias Clinical Trial: NCT03761069. Instructions. If you are interested in ... The Aplastic Anemia and MDS International Foundation. (800) 747-2820 , (301) 279-7202. 4330 East West Highway, Suite 230. ... Participant must have relapsed/refractory AML and exhausted standard available therapies known to provide clinical benefit. ...
... refractory anemia with myeloblastosis explanation free. What is refractory anemia with myeloblastosis? Meaning of refractory ... anemia with myeloblastosis medical term. What does refractory anemia with myeloblastosis mean? ... Looking for online definition of refractory anemia with myeloblastosis in the Medical Dictionary? ... refractory anaemia with myeloblastosis. (redirected from refractory anemia with myeloblastosis) refractory anaemia with ...
... of children had anemia as presenting feature. Abd el Dayem et al (3) studied 25 children with refractory iron deficiency anemia ... Anti-transglutaminase antibodies IgG (tTG) showed a value of 134 U/ml (Normal =3 U/ml). In view of refractory anemia with high ... A 10-year-old male child came to our out-patient department (OPD) for evaluation of refractory anemia. He had received adequate ... In our patient the only presenting feature was refractory anemia, the patient did not have any gastrointestinal symptoms. ...
Refractory Anemia. 12/2012. Historical perspectives on myelodysplastic syndromes.. 4/2009. Absence of frataxin mutations in ... Neither serum ferritin nor the number of red blood cell transfusions affect overall survival in refractory anemia with ringed ...
One third of patients have disease that is refractory to immunosuppression, with persistent, severe cytopenia and a profound ... Severe aplastic anemia, which is characterized by immune-mediated bone marrow hypoplasia and pancytopenia, can be treated ... We conducted a phase 2 study involving patients with aplastic anemia that was refractory to immunosuppression to determine ... Severe aplastic anemia, which is characterized by immune-mediated bone marrow hypoplasia and pancytopenia, can be treated ...
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Detection of an MPLW515 mutation in a case with features of both essential thrombocythemia and refractory anemia with ringed ... MalaCards for autosomal recessive pyridoxine-refractory sideroblastic anemia 2 - The Weizmann Institute of Science GeneCards ...
2018 Refractory Anemia with Excess Blasts Global Clinical Trials Review, H1, 2018 Summary GlobalDatas clinical trial report ... Refractory Anemia with Excess Blasts Global Clinical Trials Review, H1, ... Proportion of Refractory Anemia With Excess Blasts to Oncology Clinical Trials*Table Figure 8: Proportion of Refractory Anemia ... "Refractory Anemia with Excess Blasts Global Clinical Trials Review, H1, 2018 provides an overview of Refractory Anemia With ...
... duration of aplastic anemia diagnosis, and primary refractory disease vs relapsed refractory disease were not predictive of ... Eltrombopag and improved hematopoiesis in refractory aplastic anemia. N Engl J Med 2012;367(1):11-19. ... Describe the initial efficacy of eltrombopag in patients with refractory severe aplastic anemia, based on an open-label study. ... Eltrombopag is efficacious in a subset of patients with aplastic anemia refractory to immunosuppressive therapy, with frequent ...
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  • Iron-refractory IDA (IRIDA) has recently been described as an inherited cause of IDA due to loss-of-function mutations in the TMPRSS6 gene. (aappublications.org)
  • abstract = "TMPRSS6 variants that affect protein function result in impaired matriptase-2 function and consequently uninhibited hepcidin production, leading to iron refractory iron deficiency anemia (IRIDA). (vumc.nl)
  • Reactivation of human parvovirus B19 is exceptional and characteristic of immunosuppression, with anaemia being the predominant manifestation although pancytopenia and thrombotic microangiopathy may also occur. (ejcrim.com)
  • Antibodies that attach to and destroy lymphocytes.This may be used clinically by injection into a vein, for example, in aplastic anaemia or in other conditions where the body's immune system is being harmful. (leukaemia.org.au)
  • Anemias can be categorized into three main pathophysiologies: (1) erythrocyte loss (hemorrhage, third-space bleeds), (2) accelerated erythrocyte destruction (hemolytic anemias), and (3) decreased production (reticulocytopenia). (clinicaladvisor.com)
  • He was refractory to discontinuation of immunosuppresants, rituximab and donor lymphocyte infusions. (nature.com)
  • Although two recent randomized placebo-controlled trials of rituximab (RTX) failed to demonstrate efficacy in systemic lupus erythematosus (SLE), clinicians continue to use off-label RTX for cases refractory to current treatments. (hindawi.com)
  • Haemolytic anaemia in early infancy is often a result of blood group incompatibility, hereditary red cell morphological disorders such as congenital spherocytosis and elliptocytosis, and in certain Mediterranean and Asian regions, red cell enzyme abnormalities, including glucose-6-phosphate dehydrogenase deficiency. (bmj.com)
  • An important gene associated with Macrocytic Anemia, Refractory, Due to 5q Deletion, Somatic is RPS14 (Ribosomal Protein S14), and among its related pathways/superpathways are Regulation of degradation of deltaF508 CFTR in CF and Hematopoietic cell lineage . (malacards.org)
  • If they are large, it is macrocytic anemia while if they are normal sized, it is normocytic anemia . (wikipedia.org)