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.
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)
Bone Marrow Transplantation
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.
Complement Inactivator Proteins
Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.
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.
C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.
Complement System Proteins
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
Anemia, Hemolytic, Autoimmune
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)
Blood Cell Count
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)
A group of genetically identical cells all descended from a single common ancestral cell by mitosis in eukaryotes or by binary fission in prokaryotes. Clone cells also include populations of recombinant DNA molecules all carrying the same inserted sequence. (From King & Stansfield, Dictionary of Genetics, 4th ed)
A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.
A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.
Complement C3-C5 Convertases
Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.
Glucosephosphate Dehydrogenase Deficiency
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
Anemia, Sickle Cell
Deliberate prevention or diminution of the host's immune response. It may be nonspecific as in the administration of immunosuppressive agents (drugs or radiation) or by lymphocyte depletion or may be specific as in desensitization or the simultaneous administration of antigen and immunosuppressive drugs.
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
Pregnancy Complications, Hematologic
Bone Marrow Cells
A predominantly X-linked recessive syndrome characterized by a triad of reticular skin pigmentation, nail dystrophy and leukoplakia of mucous membranes. Oral and dental abnormalities may also be present. Complications are a predisposition to malignancy and bone marrow involvement with pancytopenia. (from Int J Paediatr Dent 2000 Dec;10(4):328-34) The X-linked form is also known as Zinsser-Cole-Engman syndrome and involves the gene which encodes a highly conserved protein called dyskerin.
Graft vs Host Disease
Complement Membrane Attack Complex
A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.
Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.
Graft vs Host Reaction
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.
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)
A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.
Hepatitis, Viral, Human
Parvovirus B19, Human
Fanconi Anemia Complementation Group Proteins
Anemia, Refractory, with Excess of Blasts
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.
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.
I Blood-Group System
Identification of the major histocompatibility antigens of transplant DONORS and potential recipients, usually by serological tests. Donor and recipient pairs should be of identical ABO blood group, and in addition should be matched as closely as possible for HISTOCOMPATIBILITY ANTIGENS in order to minimize the likelihood of allograft rejection. (King, Dictionary of Genetics, 4th ed)
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.
Separation of one or more kinds of cells from whole blood with the return of other blood cell constituents to the patient or donor. This is accomplished with an instrument that uses centrifugation to separate the cells into different layers based on the differences in cell density (displacement) or drag coefficients in a current (elutriation). The procedure is commonly used in adoptive transfer to isolate NK cells, lymphocytes, or monocytes.
Toxic, volatile, flammable liquid hydrocarbon byproduct of coal distillation. It is used as an industrial solvent in paints, varnishes, lacquer thinners, gasoline, etc. Benzene causes central nervous system damage acutely and bone marrow damage chronically and is carcinogenic. It was formerly used as parasiticide.
Colony-Forming Units Assay
Equine Infectious Anemia
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.
A phosphorus-oxygen lyase found primarily in BACTERIA. The enzyme catalyzes the cleavage of a phosphoester linkage in 1-phosphatidyl-1D-myo-inositol to form 1D-myo-inositol 1,2-cyclic phosphate and diacylglycerol. The enzyme was formerly classified as a phosphoric diester hydrolase (EC 126.96.36.199) and is often referred to as a TYPE C PHOSPHOLIPASES. However it is now known that a cyclic phosphate is the final product of this enzyme and that water does not enter into the reaction.
Granulocyte Colony-Stimulating Factor
A glycoprotein of MW 25 kDa containing internal disulfide bonds. It induces the survival, proliferation, and differentiation of neutrophilic granulocyte precursor cells and functionally activates mature blood neutrophils. Among the family of colony-stimulating factors, G-CSF is the most potent inducer of terminal differentiation to granulocytes and macrophages of leukemic myeloid cell lines.
Ham's test is occasionally positive in aplastic anemia. Ham, Thomas H. (1937). "Chronic Hemolytic Anemia with Paroxysmal ... The Ham test is a blood test used in the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). Patient red blood cells (RBCs ... MedlinePlus Encyclopedia: Ham test "Paroxysmal Nocturnal Hemoglobinuria - PNH". ARUP. Ferri, Fred F. (2015). Ferri's Clinical ... are placed in mild acid; a positive result (increased RBC fragility) indicates PNH or congenital dyserythropoietic anemia. This ...
John Vivian Dacie
... the relationship between paroxysmal nocturnal hemoglobinuria and bone marrow failure syndromes like aplastic anemia. He was ... "Historical aspects of paroxysmal nocturnal haemoglobinuria: 'defining the disease'". British Journal of Haematology. 117 (1): 3 ... His main achievements concerned the Hemolytic anemias, a field in which he was a world leader. He discovered and named ...
It does not appear to change the risk of blood clots, myelodysplastic syndrome, acute myelogenous leukemia, or aplastic anemia ... 2009). "Paroxysmal nocturnal hemoglobinuria". Hematology: Basic Principles and Practice: 385-395.CS1 maint: multiple names: ... Eculizumab, sold under the brand name Soliris among others, is a medication used to treat paroxysmal nocturnal hemoglobinuria ( ... Eculizumab is used to treat atypical hemolytic uremic syndrome (aHUS) and paroxysmal nocturnal hemoglobinuria (PNH). For people ...
... candidate autoantigen in acquired aplastic anemia patients harboring a minor population of paroxysmal nocturnal hemoglobinuria- ...
2003). "The spectrum of PIG-A gene mutations in aplastic anemia/paroxysmal nocturnal hemoglobinuria (AA/PNH): a high incidence ... 1994). "Paroxysmal nocturnal haemoglobinuria (PNH) is caused by somatic mutations in the PIG-A gene". EMBO J. 13 (1): 110-7. ... Paroxysmal nocturnal hemoglobinuria, an acquired hematologic disorder, has been shown to result from somatic mutations in this ... 1994). "Genomic organization of the X-linked gene (PIG-A) that is mutated in paroxysmal nocturnal haemoglobinuria and of a ...
Familial hemophagocytic syndrome Aplastic anemia Gaucher's disease Metastatic carcinoma of bone Multiple Myeloma Overwhelming ... Leukemia Leishmaniasis Severe folate or vitamin B12 deficiency Systemic lupus erythematosus Paroxysmal nocturnal hemoglobinuria ... anemia: hemoglobin < 13.5 g/dL (male) or 12 g/dL (female). leukopenia: total white cell count < 4.0 x 109/L. Decrease in all ... Alimentary toxic aleukia Copper deficiency Pernicious anemia Medication Hypersplenism Osteopetrosis Organic acidurias ( ...
... such as aplastic anemia, paroxysmal nocturnal hemoglobinuria, myelodysplasia and leukemias. This requires a bone marrow ... This can be due to bone marrow failure associated with aplastic anemia, leukemia, or chemotherapeutic agents. There can also be ...
List of conditions treated with hematopoietic stem cell transplantation
... sarcoma Choriocarcinoma Hematologic disease Phagocyte disorders Myelodysplasia Anemias Paroxysmal nocturnal hemoglobinuria (PNH ... Cooley's anemia) Anemias Aplastic anemia Diamond-Blackfan anemia Fanconi anemia Cytopenias Amegakaryocytic thrombocytopenia ... severe aplasia) Aplastic anemia Acquired pure red cell aplasia Myeloproliferative disorders Polycythemia vera Essential ...
Neal S. Young
... residing among patients with aplastic anemia. This work demonstrated an inherited pattern for apparently acquired aplastic ... Paroxysmal Nocturnal Hemoglobinuria and the Glycosylphosphatidylinositol-Linked Proteins. San Diego: Academic Press, 2000. ... Aplastic Anemia, Acquired and Inherited. Philadelphia: Saunders, 1994. Young, Neal S., ed. Viruses As Agents of Haematological ... He is primarily known for work in the pathophysiology and treatment of aplastic anemia, and is also known for his contributions ...
... patients with aplastic anemia also have clones of cells characteristic of the rare disease paroxysmal nocturnal hemoglobinuria ... Acton, Ashton (22 July 2013). Aplastic Anemia. ScholarlyEditions. p. 36. ISBN 978-1-4816-5068-7. Aplastic anaemia (AA) is a ... ISBN 0-679-42917-4. "Aplastic anaemia". Leukaemia Foundation. "December Is National Aplastic Anemia Awareness Month" (PDF). ... but in aplastic anemia, these cells are mostly gone and replaced by fat. First-line treatment for aplastic anaemia consists of ...
Paroxysmal nocturnal hemoglobinuria
... aplastic anemia, abnormal white blood cell counts and decreased platelet counts may be seen at this. In this case, anemia may ... Ham TH (1937). "Chronic haemolytic anaemia with paroxysmal nocturnal haemoglobinuria: study of the mechanism of haemolysis in ... The Dutch physician Enneking coined the term "paroxysmal nocturnal hemoglobinuria" (or haemoglobinuria paroxysmalis nocturna in ... It may develop on its own ("primary PNH") or in the context of other bone marrow disorders such as aplastic anemia ("secondary ...
Pregnancy associated Neonatal alloimmune associated Aplastic anemia Transfusion associated Pseudothrombocytopenia idiopathic ... Glanzmann's thrombasthenia Wiskott-Aldrich syndrome Acquired Disorders of adhesion Paroxysmal nocturnal hemoglobinuria Asthma ...
All of these, except paroxysmal nocturnal hemoglobinuria, are hereditary genetic disorders. Hereditary spherocytosis is a ... Fanconi anemia is a hereditary disorder or defect featuring aplastic anemia and various other abnormalities. Anemia of kidney ... Causes include: Acute blood loss Anemia of chronic disease Aplastic anemia (bone marrow failure) Hemolytic anemia A dimorphic ... Hyperanemia is a severe form of anemia, in which the hematocrit is below 10%. Refractory anemia, an anemia which does not ...
List of diseases (P)
Paronychia Paroxysmal cold hemoglobinuria Paroxysmal dystonic choreoathetosis Paroxysmal nocturnal hemoglobinuria Paroxysmal ... hereditary Pancreatoblastoma Panhypopituitarism Panic disorder Panmyelophthisis aplastic anemia Panniculitis Panophobia ... Perisylvian syndrome Peritonitis Periventricular laminar heterotopia Periventricular leukomalacia Pernicious anemia Perniola- ...
Cold autoimmune hemolytic anemia Cold agglutinin disease Paroxysmal cold hemoglobinuria (rare) Infectious mononucleosis ... Aplastic anemia Fanconi anemia Diamond-Blackfan anemia (inherited pure red cell aplasia) Acquired pure red cell aplasia ... Iron-deficiency anemia Megaloblastic anemia Vitamin B12 deficiency Pernicious anemia Folate deficiency Hemolytic anemias ( ... Paroxysmal nocturnal hemoglobinuria (rare acquired clonal disorder of red blood cell surface proteins) Direct physical damage ...
List of MeSH codes (C15)
... hemoglobinuria, paroxysmal MeSH C15.378.071.141.610 - hemolytic-uremic syndrome MeSH C15.378.071.196 - anemia, hypochromic MeSH ... anemia, aplastic MeSH C15.378.190.196.080 - anemia, hypoplastic, congenital MeSH C15.378.190.196.080.090 - anemia, diamond- ... MeSH C15.378.071.085 - anemia, aplastic MeSH C15.378.071.085.080 - anemia, hypoplastic, congenital MeSH C15.378.071.085.080.090 ... anemia, sideroblastic MeSH C15.378.190.625.460 - hemoglobinuria, paroxysmal MeSH C15.378.190.625.543 - leukemia, myeloid MeSH ...
... achondroplasia and congenital iodine deficiency Children after a severe episode of enteritis Pernicious anemia Aplastic anemia ... paroxysmal nocturnal hemoglobinuria (PNH) and acute myelogenous leukaemia (AML). Alkaline phosphatase is homodimeric enzyme, ... in patients with Chronic kidney disease and is directly associated with Erythropoiesis stimulating agent resistant anemia. ...
... or aplastic anemia Decreased production of thrombopoietin by the liver in liver failure Sepsis, systemic viral or bacterial ... thrombocytopenic purpura Hemolytic-uremic syndrome Disseminated intravascular coagulation Paroxysmal nocturnal hemoglobinuria ... Treatment of thrombotic thrombocytopenic purpura (TTP) is a medical emergency, since the associated hemolytic anemia and ... ETV6 related thrombocytopenia Fanconi anemia Filaminopathies A FYB related thrombocytopenia Glanzmann's thrombasthenia GNE ...
List of ICD-9 codes 280-289: diseases of the blood and blood-forming organs
... anemias 283.2 Hemoglobinuria due to hemolysis from external causes Paroxysmal nocturnal hemoglobinuria 284 Aplastic anemia ... 284.8 Other specified aplastic anemias 284.81 Other specified aplastic anemias 284.89 Red cell aplasia 284.9 Aplastic anemia ... anemia 285.1 Acute posthemorrhagic anemia 285.2 Anemia in chronic illness 285.21 Anemia in chronic kidney disease 285.22 Anemia ... 280 Iron deficiency anemias 280.0 Iron deficiency anemia secondary to blood loss (chronic) 280.1 Iron deficiency anemia ...
List of hematologic conditions
"Anemia of prematurity". Retrieved 2010-05-31. Aplastic anemia at Mount Sinai Hospital  at Mount Sinai Hospital "Hemolytic ... "Paroxysmal cold hemoglobinuria (PCH) e". Medline Plus. U.S. Department of Health and Human Services, National Institutes of ... Anemia is the most common disorder of the blood. There are several kinds of anemia, produced by a variety of underlying causes ... A nutritional anemia is a type of anemia that can be directly attributed to either a nutritional disorder or a nutritional ...
Paroxysmal nocturnal hemoglobinuria. *Asthma. *Samter's triad (aspirin exacerbated respiratory disease/AERD) ... Aplastic anemia. *Transfusion associated. *Pseudothrombocytopenia. *idiopathic thrombocytopenic purpura. *Gilbert's Syndrome[47 ...
Paroxysmal nocturnal hemoglobinuria. *Asthma. *Samter's triad (aspirin exacerbated respiratory disease/AERD) ... Aplastic anemia. *Transfusion associated. *Pseudothrombocytopenia. *idiopathic thrombocytopenic purpura. *Gilbert's Syndrome[52 ...
All of these, except paroxysmal nocturnal hemoglobinuria, are hereditary genetic disorders.*Hereditary spherocytosis is ... Aplastic anemia affects all kinds of blood cells. Fanconi anemia is a hereditary disorder or defect featuring aplastic ... "What Causes Anemia?". National Heart Lung and Blood Institute.. *^ a b c d e f g h i j k l m n o p q r s t u v w x y z aa Table ... Macrocytic anemia (MCV,100). Normocytic anemia (MCV 80-100). Microcytic anemia (MCV,80). ...
Red blood cell
Aplastic anemia is caused by the inability of the bone marrow to produce blood cells. Pure red cell aplasia is caused by the ... and paroxysmal nocturnal hemoglobinuria. The red blood cell membrane proteins organized according to their function: Transport ... Iron deficiency anemia is the most common anemia; it occurs when the dietary intake or absorption of iron is insufficient, and ... Pernicious anemia is an autoimmune disease wherein the body lacks intrinsic factor, required to absorb vitamin B12 from food. ...
paroxysmal nocturnal hemoglobinuria. *Microangiopathic hemolytic anemia. *Thrombotic microangiopathy *Hemolytic-uremic syndrome ... Aplastic. (mostly normo-). *Hereditary: Fanconi anemia. *Diamond-Blackfan anemia. *Acquired: Pure red cell aplasia ...
paroxysmal nocturnal hemoglobinuria. *Microangiopathic hemolytic anemia. *Thrombotic microangiopathy *Hemolytic-uremic syndrome ... Aplastic. (mostly normo-). *Hereditary: Fanconi anemia. *Diamond-Blackfan anemia. *Acquired: Pure red cell aplasia ... Microangiopathic hemolytic anemia. References. *^ a b Benz, K.; Amann, K. (May 2010). "Thrombotic microangiopathy: new ... The clinical presentation of TMA, although dependent on the type, typically includes: fever, microangiopathic hemolytic anemia ...
List of OMIM disorder codes
SLC6A3 Paroxysmal extreme pain disorder; 167400; SCN9A Paroxysmal nocturnal hemoglobinuria, somatic; 300818; PIGA Paroxysmal ... FGF10 Aplastic anemia; 609135; TERC Argininemia; 207800; ARG1 Argininosuccinic aciduria; 207900; ASL Aromatase deficiency; ... 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 ...
Aplastic anemia, Arthritis, Asbestosis, Ascending cholangitis (T / good article since 03 July 2008), Asthma (T), Atrial ... Paroxysmal nocturnal hemoglobinuria (T), Pendred syndrome, Peripheral artery disease (T), POEMS syndrome, Pyelonephritis, ... Hemolytic anemia (T), Hemolytic-uremic syndrome (T), Henoch-Schönlein purpura (T / good article since 7 April 2008), Heparin- ... Alkaptonuria (got onto DYK), Allergy, Alternating hemiplegia of childhood, Anaphylaxis (T), Anemia, Antiphospholipid syndrome, ...
Acute lymphoblastic leukemia
Infectious mononucleosis, acute myeloid leukemia, lymphoblastic lymphoma, aplastic anemia. Treatment. Chemotherapy, stem ... paroxysmal nocturnal hemoglobinuria, and Li-Fraumeni syndrome. Fewer than 5% of cases are associated with a known genetic ... Identical twin with ALL, Down syndrome, Fanconi anemia, ataxia telangiectasia, Klinefelter syndrome, high birth weight, ... These include: Down syndrome, Fanconi anemia, Bloom syndrome, X-linked agammaglobulinemia, severe combined immunodeficiency, ...
Red blood cell
Aplastic anemia is caused by the inability of the bone marrow to produce blood cells. ... and paroxysmal nocturnal hemoglobinuria. ... Iron deficiency anemia is the most common anemia; it occurs ... Pernicious anemia is an autoimmune disease wherein the body lacks intrinsic factor, required to absorb vitamin B12 from food. ... It can have several causes and can result in hemolytic anemia.. *The malaria parasite spends part of its life-cycle in red ...
Paroxysmal Nocturnal Hemoglobinuria | Aplastic Anemia & MDS International Foundation
Paroxysmal nocturnal hemoglobinuria | Aplastic Anemia & MDS International Foundation
... is a rare bone marrow failure disorder that manifests with hemolytic anemia, thrombosis and peripheral blood cytopenias. The ... Paroxysmal nocturnal hemoblobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolytic anemia, ... The Aplastic Anemia and MDS International Foundation. (800) 747-2820 , (301) 279-7202. 4330 East West Highway, Suite 230. ...
Study of High Dose Cyclophosphamide in Patients With Severe Aplastic Anemia and Paroxysmal Nocturnal Hemoglobinuria - Full Text...
Anemia. Anemia, Aplastic. Hemoglobinuria. Hemoglobinuria, Paroxysmal. Hematologic Diseases. Bone Marrow Diseases. Proteinuria. ... Study of High Dose Cyclophosphamide in Patients With Severe Aplastic Anemia and Paroxysmal Nocturnal Hemoglobinuria. The safety ... Genetic and Rare Diseases Information Center resources: Paroxysmal Nocturnal Hemoglobinuria Aplastic Anemia Myelodysplastic ... Disease Characteristics-- Acquired severe aplastic anemia or paroxysmal nocturnal hemoglobinuria Not a candidate for allogeneic ...
Development of clinical paroxysmal nocturnal haemoglobinuria in children with aplastic anaemia
Atsushi Narita; Hideki Muramatsu; Yusuke Okuno; Yuko Sekiya; Kyogo Suzuki; Motoharu Hamada; Shinsuke Kataoka; Daisuke Ichikawa; Rieko Taniguchi; Norihiro Murakami; Daiei Kojima; Eri Nishikawa; Nozomu Kawashima; Nobuhiro Nishio; Asahito Hama; Yoshiyuki Takahashi; Seiji Kojima + Author Information ...
Natural history of paroxysmal nocturnal hemoglobinuria clones in patients presenting as aplastic anemia<...
Natural history of paroxysmal nocturnal hemoglobinuria clones in patients presenting as aplastic anemia. European Journal of ... Natural history of paroxysmal nocturnal hemoglobinuria clones in patients presenting as aplastic anemia. / Pu, Jeffrey; Mukhina ... Natural history of paroxysmal nocturnal hemoglobinuria clones in patients presenting as aplastic anemia. ... title = "Natural history of paroxysmal nocturnal hemoglobinuria clones in patients presenting as aplastic anemia", ...
Efficacy of eculizumab in paroxysmal nocturnal hemoglobinuria patients with or without aplastic anemia: prospective study of a...
Abbreviations: AA, aplastic anemia; ecu, eculizumab; PNH, paroxysmal nocturnal hemoglobinuria.. Fig. 3. Reduction of ... Patients with paroxysmal nocturnal hemoglobinuria (PNH) often have concurrent aplastic anemia (AA). This study aimed to ... Abbreviations: AA, aplastic anemia; ecu, eculizumab; PNH, paroxysmal nocturnal hemoglobinuria; ULN, upper limit of normal. ... Abbreviations: AA, aplastic anemia; LDH, lactate dehydrogenase; PNH, paroxysmal nocturnal hemoglobinuria; RBC, red blood cells ...
Aplastic Anaemia and Paroxysmal Nocturnal Haemoglobinuria - Continuous Education - Haematology at SDMC
Aplastic Anaemia and Paroxysmal Nocturnal Haemoglobinuria Saturday, April 17, 2010 at 12:23AM ... She started off with aplastic anaemia. She emphasised the important point that aplastic anaemia is a diagnosis of exclusion. ... Aplastic anaemia is thought to have an autoimmune origin, mediated by cytotoxic T lymphocytes. Currently, there are two main ... Treatment also follow almost an identical route to aplastic anaemia although the decision to try immunosuppresion as first-line ...
Safety and Efficacy of Levamisole Combined With Cyclosporine A in Patients With Classic Paroxysmal Nocturnal Hemoglobinuria -...
Hemoglobinuria. Urination Disorders. Thrombosis. Anemia. Anemia, Aplastic. Hemoglobinuria, Paroxysmal. Pancytopenia. Anemia, ... Hemoglobinuria, Paroxysmal Proteinuria Urination Disorders Thrombosis Bone Marrow Failure Aplastic Anemia, Anemia, Hemolytic ... Genetic and Rare Diseases Information Center resources: Aplastic Anemia Paroxysmal Nocturnal Hemoglobinuria Myelodysplastic ... Paroxysmal nocturnal hemoglobinuria is an acquired chronic hemolytic anemia,this study is designed to evaluate the safety and ...
Paroxysmal Nocturnal Hemoglobinuria (PNH) | Memorial Sloan Kettering Cancer Center
... is a type of aplastic anemia. PNH is a disease of adulthood, but has been described in children as well. Memorial Sloan ... Paroxysmal nocturnal hemoglobinuria (PNH) is a type of acquired aplastic anemia. It is caused by a mutation that happens in a ... In addition to acquired aplastic anemia, children and young adults with PNH may have other complications. For example, red ... Fanconi Anemia and Other Inherited Bone Marrow Failure Syndromes * Thalassemia, Sickle Cell Anemia, and Other Inherited ...
Basiliximab #2: In-Vivo Activated T-Cell Depletion to Prevent Graft-Versus Host Disease (GVHD) After Nonmyeloablative...
Hemoglobinuria. Anemia, Aplastic. Hemoglobinuria, Paroxysmal. Neoplasms by Histologic Type. Neoplasms. Neoplasms, Plasma Cell. ... Aplastic Anemia Paroxysmal Nocturnal Hemoglobinuria Hodgkin Lymphoma Chronic Myeloid Leukemia Chronic Myeloproliferative ... Anemia, Aplastic Hemoglobinuria, Paroxysmal Drug: Basiliximab Phase 2 ... Patients with aplastic anemia must have marrow cellularity ≤ 10% plus 2 of the following:. *Absolute granulocyte count ,500/mm3 ...
Paroxysmal nocturnal hemoglobinuria clones in children with acquired aplastic anemia: A multicentre study - Fingerprint -...
Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic...
... cells and leucocyte subset telomere lengths in paediatric aplastic anaemia (AA) is unknown. Among 22 children receiving ... The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) ... Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic ... The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) cells and leucocyte subset telomere lengths in paediatric ...
Thrombocytopenia in Pregnancy: Practice Essentials, Definition and Clinical Manifestations, Etiologic Classification
Aplastic anemia. * Paroxysmal nocturnal hemoglobinuria. * Infection. * Bone marrow infiltration (hematologic malignancy, ... A retrospective study by Shin et al indicated that in pregnant women with aplastic anemia, obstetric and disease complications ... The study, which included 61 patients with aplastic anemia, found that in women with severe thrombocytopenia, the incidence of ... Association of severe thrombocytopenia and poor prognosis in pregnancies with aplastic anemia. PLoS One. 2014. 9(7):e103066. [ ...
Research at Johns Hopkins Medicine
Aplastic Anemia: Practice Essentials, Background, Etiology
Aplastic anemia is a syndrome of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia. Although ... link between aplastic anemia and paroxysmal nocturnal hemoglobinuria is suggested by a high frequency of aplastic anemia ... Incidence of aplastic anemia in Bangkok. The Aplastic Anemia Study Group. Blood. 1991 May 15. 77(10):2166-8. [Medline]. ... encoded search term (Aplastic%20Anemia) and Aplastic Anemia What to Read Next on Medscape. Medscape Consult. ...
What Are the Signs and Symptoms of Aplastic Anemia? | HOA - Hematology-Oncology Associates of CNY
... and platelets cause most of the signs and symptoms of aplastic anemia. Signs and Symptoms of Low Blood Cell Counts Red Blood ... Paroxysmal Nocturnal Hemoglobinuria. Some people who have aplastic anemia have a condition called paroxysmal (par-ok-SIZ-mal) ... Aplastic anemia can cause signs and symptoms that arent directly related to low blood cell counts. Examples include nausea ( ... What Are the Signs and Symptoms of Aplastic Anemia?. Lower than normal numbers of red blood cells, white blood cells, and ...
A PATHOGENETIC LINK BETWEEN APLASTIC-ANEMIA AND PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA IS SUGGESTED BY A HIGH-FREQUENCY OF...
Efficacy of Low Dose Combined Chemotherapy for Patients with Relapsed and Refractory Aplastic Anemia-Paroxysmal Nocturnal...
Hemoglobinuria, Paroxysmal / Hemolysis / Anemia, Aplastic Language: Chinese Journal: Journal of Experimental Hematology Year: ... for patients with relapsed and refractory aplastic anemia-paroxysmal nocturnal hemoglobinuria（AA-PNH） syndrome, and to analyze ... of Low Dose Combined Chemotherapy for Patients with Relapsed and Refractory Aplastic Anemia-Paroxysmal Nocturnal Hemoglobinuria ... Full text: Available Index: WPRIM (Western Pacific) Main subject: Anemia, Refractory / Humans / Retrospective Studies / ...
Relative increase of granulocytes with a paroxysmal nocturnal hemoglobinuria phenotype is a common feature of aplastic anemia...
Relative increase of granulocytes with a paroxysmal nocturnal hemoglobinuria phenotype is a common feature of aplastic anemia ... Relative increase of granulocytes with a paroxysmal nocturnal hemoglobinuria phenotype is a common feature of aplastic anemia ... Relative increase of granulocytes with a paroxysmal nocturnal hemoglobinuria phenotype is a common feature of aplastic anemia ... Relative increase of granulocytes with a paroxysmal nocturnal hemoglobinuria phenotype is a common feature of aplastic anemia ...
Monosomy 7 in patients with aplastic anemia and paroxysmal nocturnal hemoglobinuria with evolution into acute myeloid leukemia....
We report two cases of Thai patients with aplastic anemia/paroxysmal nocturnal hemoglobinuria (AA/PNH) who subsequently ... Monosomy 7 in patients with aplastic anemia and paroxysmal nocturnal hemoglobinuria with evolution into acute myeloid leukemia ... Hemoglobinuria, Paroxysmal / Anemia, Aplastic / Karyotyping Type of study: Case report Clinical aspect: Diagnosis Language: ... Monosomy 7 in patients with aplastic anemia and paroxysmal nocturnal hemoglobinuria with e ...
Paroxysmal Nocturnal Hemoglobinuria (PNH) - NORD (National Organization for Rare Disorders)
Further evidence of the relation to aplastic anemia is shown in that up to 40% of PNH patients have a history of AA and up to ... What is Paroxysmal Nocturnal Hemoglobinuria (PNH)?. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematological ... Ham TH., "Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria. A study of the mechanism of hemolysis in relation ... Paroxysmal Nocturnal Hemoglobinuria (PNH). Print. Introduction. Welcome to the NORD Physician Guide to Paroxysmal Nocturnal ...
The Aplastic Anaemia Trust | Pages | Types of rare bone marrow failure
The Aplastic Anaemia Trust is the only charity in the UK focused on raising funds for research into aplastic anaemia and other ... Aplastic anaemia can also lead to paroxysmal nocturnal haemoglobinuria (PNH), and vice versa, as in our experience some ... Jerome shares his aplastic anaemia story so far. Read about Jeromes aplastic anaemia journey so far ... What is aplastic anaemia?. Aplastic means your bone marrow is failing to produce enough of ALL essential blood cells ...
All You Need To Know About Aplastic Anemia Or Bone Marrow Failure (Hypoplastic Anemia) | HubPages
This is anemia resulting from failure to produce the formed elements of blood by the bone marrow. Morphologically, in the vast ... and paroxysmal nocturnal hemoglobinuria. Acute myeloid leukemia may be mistaken for alastic anemia in the initial stages, but ... Aplastic anemia is a rare disorder contributing to 1-2% of the total number of anemia cases. Males suffer twice as many as ... All You Need To Know About Aplastic Anemia Or Bone Marrow Failure (Hypoplastic Anemia). Updated on January 20, 2014 ...
XmnI polymorphism is associated with fetal hemoglobin levels in hypoplastic syndromes
Our objectives were to examine acquired HbF increases in aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) ... Key words: Polymorphism (Genetics). Aplastic anemia. Paroxysmal hemoglobinuria. Fetal hemoglobin. Polymerase chain reaction. ... Our objectives were to examine acquired HbF increases in AA and paroxysmal nocturnal hemoglobinuria (PNH) patients, and to ... Nossos objetivos foram de examinar elevações adquiridas na HbF em pacientes com anemia aplástica (AA) e hemoglobinúria ...
Paroxysmal nocturnal hemoglobinuria: MedlinePlus Genetics
Paroxysmal nocturnal hemoglobinuria is an acquired disorder that leads to the premature death and impaired production of blood ... The spectrum of PIG-A gene mutations in aplastic anemia/paroxysmal nocturnal hemoglobinuria (AA/PNH): a high incidence of ... people who have been treated for another blood disease called aplastic anemia may develop paroxysmal nocturnal hemoglobinuria. ... medlineplus.gov/genetics/condition/paroxysmal-nocturnal-hemoglobinuria/ Paroxysmal nocturnal hemoglobinuria. ...
Detection of PNH red cell population in a cohort of patients by using the gel test in a resource constrained country: a cross...
Paroxysmal nocturnal hemoglobinuria; ID-PNH; Aplastic anemia. Paroxysmal Nocturnal Haemoglobinuria (PNH) is an acquired ... Paroxysmal nocturnal hemoglobinuria clones in severe aplastic anemia patients treated with horse anti-thymocyte globulin plus ... Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria: Certain immunological aspects of the hemolytic mechanism ... Our study shows the presence of PNH red cells in 5 patients with Aplastic Anemia and 2 patients with Hemolytic Anemia. The ...
Cancer Research & Clinical Trials | Cleveland Clinic
Aplastic Anemia *Paroxysmal Nocturnal Hemoglobinuria *Myelodysplastic Syndrome *Large Granular Lymphocytic Leukemia *Lung ... Our laboratory investigates pathogenesis of several hematopoietic disorders including aplastic anemia, paroxysmal nocturnal ... aplastic anemia has contributed to the discovery of frequent clonal events in the otherwise considered benign aplastic anemia ... Further evidence that paroxysmal nocturnal haemoglobinuria is a disorder of defective cell membrane lipid rafts. J. Cell Mol ...
Our Experts | Sidney Kimmel Cancer Center
Ham test - Wikipedia
Hams test is occasionally positive in aplastic anemia. Ham, Thomas H. (1937). "Chronic Hemolytic Anemia with Paroxysmal ... The Ham test is a blood test used in the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). Patient red blood cells (RBCs ... MedlinePlus Encyclopedia: Ham test "Paroxysmal Nocturnal Hemoglobinuria - PNH". ARUP. Ferri, Fred F. (2015). Ferris Clinical ... are placed in mild acid; a positive result (increased RBC fragility) indicates PNH or congenital dyserythropoietic anemia. This ...
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Pure Red Cel2
- Acquired bone marrow failure diseases include aplastic anemia, myelodysplasia, paroxysmal nocturnal hemoglobinuria and pure red cell aplasia. (army.mil)
- Distinct syndromes are, therefore, defined by the specific cells affected and include pure red cell aplasia (PRCA), amegakaryocytic thrombocytopenic purpura, aplastic anemia (AA), and myelodysplastic syndrome. (frontiersin.org)
- Individuals affected with acquired aplastic anemia are also at risk that it will evolve into another similar disorder known as myelodysplasia. (rarediseases.org)
- A serious complication of aplastic anemia (AA) is its evolution to clonal hematologic diseases such as myelodysplasia (MDS) and leukemia, which is usually associated with the appearance of a cytogenetic abnormality in bone marrow cells. (bloodjournal.org)
- Evolution of acquired severe aplastic anaemia to myelodysplasia and subsequent leukaemia in adults. (springer.com)
- Conditions of the bone marrow in which not enough blood cells are produced, such as aplastic anemia, myelodysplasia, and paroxysmal nocturnal hemoglobinuria. (dana-farber.org)
- A number of malignant diseases may present with aplastic anemia, including: myelodysplasia, myelofibrosis, paroxsymal nocturnal hemoglobinuria (PNH), acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), Hodgkin disease, and various solid tumors. (clinicalpainadvisor.com)
- Memorial Sloan Kettering experts are leaders in diagnosing and treating pediatric blood disorders, including inherited bone marrow failure syndromes , acquired aplastic anemia , and hemoglobinopathies . (mskcc.org)
- The complimentary symposium will provide an in-depth and up-to-date review of research related to the biology, prognosis, natural history, and therapeutic management of patients with aplastic anemia, paroxysmal nocturnal hemoglobinuria (PNH), myelodysplastic syndromes (MDS), and acute myeloid leukemia (AML). (clevelandclinicmeded.com)
- Inherited bone marrow failure syndromes (IBMFS) and acquired aplastic anemia (AA) are life‐threatening marrow failure disorders. (ingentaconnect.com)
- Common causes of B 12 deficiency include malabsorption syndromes (such as celiac sprue or tropical sprue), any prior intestinal surgeries (including gastric bypass and other stomach surgeries), inflammatory bowel disease (IBD), Diphyllobothrium latum infection, pancreatic insufficiency, and pernicious anemia. (dummies.com)
- Sir Dacie is credited with characterizing the relationship between paroxysmal nocturnal hemoglobinuria and bone marrow failure syndromes like aplastic anemia. (wikipedia.org)
- ESAs are no longer recommended for the treatment of cancer-related anemia associated with solid tumors or hematologic malignancies other than myelodysplastic syndromes (MDS). (medworm.com)
- This may occur as the result of inherited abnormalities as seen in syndromes like Fanconi anemia, dyskeratosis congenital, and Shwachman-Diamond syndrome, or may be an acquired phenomena ( 3 ). (frontiersin.org)
- Aplastic anaemia is thought to have an autoimmune origin, mediated by cytotoxic T lymphocytes. (harishematology.com)
- Clinical and laboratory observations suggest that acquired aplastic anemia is an autoimmune disease. (medscape.com)
- It is believed that PNH arises in the setting of autoimmune acquired aplastic anemia and bone marrow failure. (rarediseases.org)
- Cyclosporin A (CsA) is used to prevent rejection in transplantation and to treat autoimmune and hematologic diseases such as aplastic anemia. (springer.com)
- Aplastic anemia that occurs in pregnancy may be related to an autoimmune problem - your immune system may attack your bone marrow during pregnancy. (mayoclinic.org)
- Pernicious anemia is caused by a lack of intrinsic factor, usually due to an autoimmune condition in which the body forms antibodies against the gastric parietal cells and can destroy them. (dummies.com)
- But their story line also helps to bring awareness to the YouTuber's extremely rare autoimmune disease called aplastic anemia. (intouchweekly.com)
- Predictors of refractoriness to therapy and healthcare resource utilization in 378 patients with primary autoimmune hemolytic anemia from 8 Italian Reference Centers. (amedeo.com)
- Other considerations include: aplastic anemia associated with pregnancy, ionizing radiation, histiocytic disorders (hemophagocytic lymphohistiocytosis), osteopetrosis, storage diseases, Pearson syndrome (mitochrondial deletions), rheumatologic and autoimmune diseases, anorexia nervosa and acquired vitamin and nutritional deficiencies. (clinicalpainadvisor.com)
- Some inherited genes cause diseases such as Tay-Sachs or sickle cell anemia, while mutations in genes can cause genetic disorders, such as Down syndrome. (sutterhealth.org)
- Although bone marrow failure can occur secondary to other disorders, most aplastic anemia is due to the immune system mistakenly targeting the bone marrow (autoimmunity). (rarediseases.org)
- High MCV values are also commonly observed in anemias accompanying myelodysplastic disorders. (clinicaladvisor.com)
- The most common inherited bone marrow failure disorders include Fanconi anemia, dyskeratosis congenita, Shwachman-Diamond syndrome, and Diamond-Blackfan anemia. (army.mil)
- Paroxysmal nocturnal hemoglobinuria (PNH) often develops secondary to other bone marrow failure (BMF) disorders, especially aplastic anemia (AA). (bioportfolio.com)
- An expanded PNH clone is also found in up to 70% of patients with acquired aplastic anemia (AA), demonstrating a pathophysiological link between these disorders. (cancertherapyadvisor.com)
Severe aplastic anaemia5
- At the young age of 17 on the 12th December 30 years ago, I was diagnosed with severe aplastic anaemia. (theaat.org.uk)
- The impact of Asian descent on the incidence of acquired severe aplastic anaemia in children. (thedoctorsdoctor.com)
- Previous studies have suggested an increased incidence of acquired severe aplastic anaemia in Asian populations. (thedoctorsdoctor.com)
- This study shows that Asian children have an increased incidence of severe aplastic anaemia possibly as a result of a genetic predisposition. (thedoctorsdoctor.com)
- Unmanipulated haploidentical transplantation conditioning with busulfan, cyclophosphamide and anti-thymoglobulin for adult severe aplastic anaemia. (amedeo.com)
Children with acquired aplastic anemia1
- Myelodysplastic syndrome and acute myelogenous leukemia as a late clonal complication in children with acquired aplastic anemia. (springer.com)
- PNH patients are anemic due to intravascular hemolysis and an underlying marrow dysfunction, usually characteristic of aplastic anemia. (rarediseases.org)
- PNH is a debilitating and life-threatening disorder characterized by chronic intravascular hemolysis and thrombophilia 1,2 and can also occur in the setting of bone marrow hypoplasia characteristic of aplastic anemia. (rarediseases.org)
- Nocturnal hemoglobinuria and paroxysms of hemolysis are due to the greater activation of complement that can occur from nocturnally absorbed liposaccharide or infection, trauma, surgery, pregnancy etc. (rarediseases.org)
- Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria. (semanticscholar.org)
- In addition to B 12 and folic acid deficiencies, other causes of macrocytic anemias include alcoholism, hypothyroidism, chronic liver disease, and hemolysis. (dummies.com)
- ABSTRACT: Paroxysmal nocturnal hemoglobinuria is an ultra-rare disorder characterized by hemolysis, thrombosis, and pancytopenia. (bcmj.org)
- We recommend that all patients with proven aplastic anemia or unexplained hemolysis, thrombosis, or persistent pancytopenia be tested for paroxysmal nocturnal hemoglobinuria, but that patients with cytopenia involving only one or two cell lines not be tested. (bcmj.org)
- Paroxysmal nocturnal hemoglobinuria (PNH) is an ultra-rare, acquired blood disorder that is characterized by hemolysis, thrombosis, and pancytopenia (anemia, leukopenia, and thrombocytopenia) due to bone marrow failure. (bcmj.org)
- A retrospective study by Shin et al indicated that in pregnant women with aplastic anemia, obstetric and disease complications are more prevalent in those with severe thrombocytopenia than in those with nonsevere thrombocytopenia. (medscape.com)
- The study, which included 61 patients with aplastic anemia, found that in women with severe thrombocytopenia, the incidence of transfusion during pregnancy or the postpartum period (72.7% and 45%, respectively) was greater than in those with nonsevere thrombocytopenia (15.4% and 2.7%, respectively). (medscape.com)
- Megakaryocytes, myeloid elements and erythroblasts are progressively wiped out resulting in thrombocytopenia, neutropenia and anemia as the disease evolves. (hubpages.com)
- Symptoms are dependent on the severity of the anemia, leukopenia, and thrombocytopenia. (rarediseases.org)
- Serious and fatal blood dyscrasias (aplastic anemia, hypoplastic anemia, thrombocytopenia and granulocytopenia) are known to occur after the administration of chloramphenicol. (drugs.com)
- It is therefore the combination of anaemia, leukopenia, and thrombocytopenia. (bmj.com)
- Depleted cell lines lead to symptoms of fatigue from anemia, bleeding symptoms from thrombocytopenia, or infections from neutropenia. (oncologynurseadvisor.com)
- A new onset of thrombocytopenia and microangiopathic hemolytic anemia in the healthcare setting: A challenge for diagnosis. (amedeo.com)
- Children may present with fatigue and pallor that is the result of anemia, bruising, and easy bleeding that is the result of thrombocytopenia and fever and infections that are the result of neutropenia. (clinicalpainadvisor.com)
- Bone marrow biopsy is essential in all cases, since it helps to rule out conditions like acute leukemia, lymphomas, multiple myeloma, myelofibrosis, which may superficially resemble aplastic anemia. (hubpages.com)
- Dameshek W (1969) Forward and a proposal for considering paroxysmal nocturnal hemoglobinuria (PNH) as a "candidate" myeloproliferative disorder. (springer.com)
- Fanconi's anemia is a rare, inherited disease that leads to aplastic anemia. (mayoclinic.org)
- Autologous black and Afrikaner population groups of SA are at greater risk of haematopoietic stem cell transplantation, on the other hand, denotes inheriting Fanconi's anaemia owing to the genetic founder effect of the collection of the patient's own stem cel s prior to high-dose the mutant Fanconi-associated genes. (who.int)
- Chromosome fragility test and detailed family history in patients less than 40 years old to investigate for Fanconi's anemia. (oncologynurseadvisor.com)
- Also included is an important international consensus document on treatment, and a final section concentrates on the inherited syndrome Fanconi's anemia. (whsmith.co.uk)
- Fanconi's Anemia: 17. (whsmith.co.uk)
- 20. Genetic correction of Fanconi's anemia J. M. Liu. (whsmith.co.uk)
- Objective:To investigate the natural history of paroxysmal nocturnal hemoglobinuria (PNH) clones in patients with acquired aplastic anemia (AA). (elsevier.com)
- Due to therapy options and the relevance of GPI-deficient clones for prognosis in aplastic anaemia detection of PNH is gaining importance. (springer.com)
- Detection of paroxysmal nocturnal hemoglobinuria clones to exclud. (ingentaconnect.com)
Guide to Paroxysmal Nocturnal Hemo1
- Welcome to the NORD Physician Guide to Paroxysmal Nocturnal Hemoglobinuria (PNH). (rarediseases.org)
Adult patients with severe aplastic anemia1
- Successful outcomes of second hematopoietic stem cell transplantation with total nodal irradiation and ATG conditioning for graft failure in adult patients with severe aplastic anemia. (amedeo.com)
- a positive result (increased RBC fragility) indicates PNH or congenital dyserythropoietic anemia. (wikipedia.org)
- In contrast, anemias resulting from accelerated red cell destruction suggest congenital, immune-mediated, or toxic pathophysiologies that, as a group, will exhibit a less predictable response to red cell transfusion and may require additional disease-specific therapies. (clinicaladvisor.com)
- and, less commonly, anemias associated with chronic inflammatory conditions, genetic determinants for Hb C, congenital defects in copper metabolism, some forms of sideroblastic anemia, and other conditions. (clinicaladvisor.com)
- Congenital sideroblastic anemia in a female. (amedeo.com)
- Congenital dyserythropoietic anemia type 1: a case with novel compound heterozygous mutations in the C15orf41 gene. (amedeo.com)
Patients with paroxysmal nocturnal hemo2
- Patients with malignant and non-malignant hematologic diseases including severe aplastic anemia (SAA), paroxysmal nocturnal hemoglobinuria (PNH), myelodysplastic syndrome (MDS), acute and chronic leukemias, Hodgkin's and non-Hodgkin's lymphoma and multiple myeloma can now be cured by allogeneic bone marrow transplantation (BMT). (knowcancer.com)
- Patients with myelodysplastic syndrome usually will present with anemia and lymphoma with lymphadenopathy. (aafp.org)
- A bone marrow or cord blood transplant may be the best treatment option or the only potential for a cure for patients with leukemia, lymphoma, sickle cell anemia and many other diseases. (bethematch.org)
- Among the polymorphic genetic markers of the b -globin gene cluster, the XmnI polymorphism, a common C ® T variation at position 158 of the G g globin gene, and a set of sequence variations in the 5' hypersensitive site (HS2) of the LCR have been associated with increased HbF levels in normal individuals, and in b -thalassemia and sickle cell anemia patients. (scielo.br)
- Researchers are looking into the benefits of stem cell transplants to treat many forms of cancer, sickle cell disease and certain rare types of anemia. (nortonhealthcare.com)
- Hydroxyurea prevents onset and progression of albuminuria in children with sickle cell anemia. (amedeo.com)
- Striking dyserythropoiesis in sickle cell anemia following an aplastic crisis. (amedeo.com)
- Progressive Loss of Brain Volume in Children with Sickle Cell Anemia and Silent Cerebral Infarct: A Report from the Silent Cerebral Infarct Transfusion (SIT) Trial. (amedeo.com)
- Prognostic factors of disease severity in infants with sickle cell anemia: A comprehensive longitudinal cohort study. (amedeo.com)
Transplantation for Severe Aplastic Anemia2
- Impact of ABO incompatibility on outcomes after haploidentical hematopoietic stem cell transplantation for severe aplastic anemia. (amedeo.com)
- Unrelated cord blood transplantation for severe aplastic anemia using intensified immunoablative conditioning regimen leading to high engraftment and survival. (amedeo.com)
- The clinical presentation of patients with aplastic anemia includes symptoms related to the decrease in bone marrow production of hematopoietic cells. (medscape.com)
- [email protected]#To evaluate the clinical efficacy of low dose combined chemotherapy（LDCC） for patients with relapsed and refractory aplastic anemia-paroxysmal nocturnal hemoglobinuria（AA-PNH） syndrome, and to analyze the advantages of LDCC in the treatment of AA-PNH syndrome. (bvsalud.org)
- In general, anemias that evolve over a long interval will have a less pronounced clinical impact than anemias that develop acutely. (clinicaladvisor.com)
- Dacie JV, Lewis SM (1961) Paroxysmal nocturnal hemoglobinuria: variation in clinical severity and association with bone marrow hypoplasia. (springer.com)
- Dacie JV, Lewis SM (1972) Paroxysmal nocturnal hemoglobinuria, clinical manifestations, hematology and nature of the disease. (springer.com)
- This book takes account of the most recent findings in laboratory research and clinical trials to provide a comprehensive and up-to-date reference on the pathophysiology, epidemiology, diagnosis and treatment of acquired and inherited aplastic anemia. (whsmith.co.uk)
- Epidemiology and Clinical Features of Acquired Aplastic Anemia: 7. (whsmith.co.uk)
- The clinical severity of aplastic anemia is classified based on peripheral blood counts and results of bone marrow examinations. (clinicalpainadvisor.com)
- Several classification systems have been proposed and used to assess the clinical severity of aplastic anemia. (clinicalpainadvisor.com)
- Quantification of small clonal populations of GPI-negative cells influences clinical decisions to administer immunosuppressive therapy in marrow failure states (aplastic anemia or myelodysplastic syndrome) and to monitor minimal residual disease after allogeneic blood or marrow transplantation (BMT). (wiley.com)
Diagnosed with severe aplastic1
- That is oftentimes the cause of death of people who are diagnosed with severe aplastic anemia," she explained and added that because of the low platelets, she had lots of bruising all over her body. (intouchweekly.com)
- Paroxysmal nocturnal hemoblobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolytic anemia , thrombosis and peripheral blood cytopenias. (aamds.org)
- Aplastic anemia is a syndrome of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia (see the image below). (medscape.com)
- Paul Ehrlich introduced the concept of aplastic anemia in 1888 when he reported the case of a pregnant woman who died of bone marrow failure. (medscape.com)
- Fanconi anaemia is a complex medical condition that may lead to bone marrow failure, physical abnormalities, organ defects, and an increased risk of certain cancers. (theaat.org.uk)
- This is anemia resulting from failure to produce the formed elements of blood by the bone marrow. (hubpages.com)
- Paroxysmal Nocturnal Haemoglobinuria (PNH) is an acquired haematopoietic stem cell disorder characterized by a triad of hemolytic Anemia, bone marrow failure and thrombosis [ 1 ]. (pulsus.com)
- Acquired aplastic anemia is a rare, serious blood disorder, due to failure of the bone marrow failure to produce blood cells. (rarediseases.org)
- Stem cell transplantation , also known as bone marrow transplantation, is used to treat blood diseases such as aplastic anemia, bone marrow failure, and blood cancers, as well as to restore blood-forming cells in the body. (bumrungrad.com)
- AAMAC funds research into bone marrow failure diseases such as aplastic anemia, PNH and MDS. (aamac.ca)
- Fifteen to 20% of children with severe aplastic anemia have an inherited bone marrow failure syndrome, most often Fanconi anemia. (clinicalpainadvisor.com)
- Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disease that can present with bone marrow failure, hemolytic anemia, smooth muscle dystonias, and thrombosis. (cancertherapyadvisor.com)
- BMT has been shown to be curative in a number of debilitating hematologic diseases which may behave in a relatively indolent fashion, such as PNH and refractory anemia (RA) or refractory anemia with ringed sideroblasts (RARS). (knowcancer.com)
- Monosomy 7 in patients with aplastic anemia and paroxysmal nocturnal hemoglobinuria with evolution into acute myeloid leukemia. (bvsalud.org)
- We report two cases of Thai patients with aplastic anemia/paroxysmal nocturnal hemoglobinuria (AA/PNH) who subsequently developed acute myeloid leukemia (AML) at their terminal phase. (bvsalud.org)
- Acute myeloid leukemia may be mistaken for alastic anemia in the initial stages, but presence of splenomegaly is suggestive. (hubpages.com)
- Clonal evolution of aplastic anaemia to myelodysplastic/acute myeloid leukemia and paroxysmal nocturnal haemoglobinuria. (springer.com)
- However, it was not until 1904 that Anatole Chauffard named this disorder aplastic anemia. (medscape.com)
- Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematological disorder of the hematological stem cell, thus involving all blood cells. (rarediseases.org)
- Aplastic anemia is a rare disorder contributing to 1-2% of the total number of anemia cases. (hubpages.com)
- Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder that is caused due to the absence of certain glycosylphophatidylinositol (GPI)-anchored proteins, such as CD55 and CD59. (pulsus.com)
- Some individuals with acquired aplastic anemia also have another disorder at the same time, called paroxysmal nocturnal hemoglobinuria (PNH). (rarediseases.org)
- Some people with aplastic anemia also have a rare disorder known as paroxysmal nocturnal hemoglobinuria. (mayoclinic.org)
- Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal disorder of the hematopoietic stem cell (Hartmann and Arnold 1977) resulting in the production of blood cells which are defective in that they lack or are markedly deficient in glycan-phosphatidylinositol (GPI)-linked surface proteins (Rosse 1990a). (springer.com)
- Aplastic anemia (AA) is a disorder of hematopoietic stem cells that results in pancytopenia in the setting of a hypocellular bone marrow and normal spleen size. (oncologynurseadvisor.com)
- AA is also associated with a blood disorder called paroxysmal nocturnal hemoglobinuria (PNH). (oncologynurseadvisor.com)
- Have you been tested for aplastic anemia or a bone marrow disorder? (webmd.com)
- Aplastic anemia is a rare and potentially fatal disorder that is defined by pancytopenia with a hypocellular bone marrow. (clinicalpainadvisor.com)
Paediatric aplastic anaemia2
- The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) cells and leucocyte subset telomere lengths in paediatric aplastic anaemia (AA) is unknown. (telomerescience.com)
- It worth noting that some of the main treatment options may also apply to young patients, however they will be treated by experts specialising in paediatric aplastic anaemia. (theaat.org.uk)
- The picture of peripheral pancytopenia with aplastic bone marrow confirms the diagnosis of aplastic anemia. (hubpages.com)
- Peripheral pancytopenia with normocellular or hypercellular marrow should suggest the possibility of aleukemic leukemia, sideroblastic anemia, dyserythropoietic states, and paroxysmal nocturnal hemoglobinuria. (hubpages.com)
- In acquired aplastic anemia, an almost complete absence of hematopoietic stem cells results in low levels of red and white blood cells and platelets (pancytopenia). (rarediseases.org)
- Aplastic anemia (AA) is characterized by hypocellular marrow and peripheral pancytopenia. (bloodjournal.org)
- Aplastic anemia (AA) is a life-threatening disease characterized by hypocellular marrow and pancytopenia as a result of reduction in hematopoietic progenitor and stem cells (HSPCs). (bloodjournal.org)
- Without consideration of the patient's presenting condition and history, any disease with pancytopenia may be mistaken for aplastic anemia. (oncologynurseadvisor.com)
- The signs and symptoms of aplastic anemia are primarily those associated with pancytopenia. (clinicalpainadvisor.com)
Leads to aplastic anemia1
- IFN-γ alone leads to aplastic anemia by disrupting the generation of common myeloid progenitors and lineage differentiation. (bloodjournal.org)
- Eculizumab, an antibody to the fifth component of complement, can reduce or stop the intravascular destruction of red cells and decrease anemia in PNH patients, but has no effect on the marrow dysfunction. (rarediseases.org)
- Most cases of aplastic anemia are due to a deficiency of marrow stem cells. (hubpages.com)
- The symptoms of acquired aplastic anemia occur as a consequence of the bone marrow failing to produce enough blood cells. (rarediseases.org)
- There are a minority of MDS patients with hypoplastic or low cellularity bone marrow, as seen in acquired aplastic anemia. (rarediseases.org)
- With the introduction of immunosuppressive therapy (IST) for aplastic anemia (AA), the survival of patients who are not treated with bone marrow (BM) transplantation has improved significantly. (bloodjournal.org)
- While blood studies may detect early peripheral blood changes, such as leukopenia, reticulocytopenia, or granulocytopenia, before they become irreversible, such studies cannot be relied on to detect bone marrow depression prior to development of aplastic anemia. (drugs.com)
- Treatment for aplastic anemia may include medications, blood transfusions or a stem cell transplant, also known as a bone marrow transplant. (mayoclinic.org)
- Aplastic anemia develops when damage occurs to your bone marrow, slowing or shutting down the production of new blood cells. (mayoclinic.org)
- In aplastic anemia, the bone marrow is described in medical terms as aplastic or hypoplastic - meaning that it's empty (aplastic) or contains very few blood cells (hypoplastic). (mayoclinic.org)
- Viral infections that affect bone marrow may play a role in the development of aplastic anemia in some people. (mayoclinic.org)
- But some people with myelodysplastic syndrome have empty marrow that's difficult to distinguish from aplastic anemia. (mayoclinic.org)
- Fifty patients with severe aplastic anemia had no transfusions of blood products until just before marrow transplantation from HLA-identical family members. (annals.org)
- Outcome of hematopoietic stem cell transplantation (HCT) from HLA-matched related donor for Fanconi anemia (FA) in adolescents and adults: a retrospective study by Eastern Mediterranean Blood and Marrow Transplantation Group (EMBMT). (amedeo.com)
- Recently, Davies and Guinan proposed definitions for aplastic anemia based on peripheral blood cytopenias and bone marrow results. (clinicalpainadvisor.com)
- In this classification schema, children with severe aplastic anemia have bone marrow cellularity less than 25% of normal, and two or more of the following: peripheral blood neutrophil count less than 0.5 x 10 9 /L, or peripheral blood platelet count less than 20 x 10 9 /L, or peripheral blood reticulocyte count less than 20 x 10 9 /L. (clinicalpainadvisor.com)
- Children with nonsevere aplastic anemia have a hypocellular bone marrow without this severity of peripheral blood findings. (clinicalpainadvisor.com)
Chronic hemolytic anemia1
- Paroxysmal nocturnal hemoglobinuria is an acquired chronic hemolytic anemia,this study is designed to evaluate the safety and efficacy of Levamisole combined with cyclosporine A in patients with classic paroxysmal nocturnal hemoglobinuria. (clinicaltrials.gov)
White blood cells and pla2
- Lower than normal numbers of red blood cells, white blood cells, and platelets cause most of the signs and symptoms of aplastic anemia. (hoacny.com)
- The result is more than just anemia, but severe reduction in the other blood components such as the white blood cells and platelets. (thedoctorsdoctor.com)
Symptoms of aplastic anemia2
Treatment of aplastic anemia1
- Malignant tumors occurring after treatment of aplastic anemia. (springer.com)
Development of aplastic anemia1
- Viruses that have been linked to the development of aplastic anemia include hepatitis, Epstein-Barr, cytomegalovirus, parvovirus B19 and HIV. (mayoclinic.org)
Cause aplastic anemia2
Causes Aplastic Anemia1
- What Causes Aplastic Anemia? (hubpages.com)
- However, acquired HbF elevation has been implicated as a prognostic factor in myelodysplastic syndrome (MDS) and aplastic anemia (AA). (scielo.br)
- Aplastic anemia can be mistaken for a condition called myelodysplastic syndrome. (mayoclinic.org)
- As well as being useful in patients suspected of having paroxysmal nocturnal hemoglobinuria, PNH testing is useful in patients with aplastic anemia and myelodysplastic syndrome. (bcmj.org)
- Bryant PM, Hall SE, Cole JS, Greenberg CS, Rosse WF (1988) Marked sensitivity of paroxysmal nocturnal hemoglobinuria (PNH) platelets to thrombin: relationship to complement activation. (springer.com)
- Devine DV, Siegel RS, Rosse WF (1987a) Interactions of the platelets in paroxysmal nocturnal hemoglobinuria with complement. (springer.com)
- Flow cytology of peripheral blood helps establish or rule out paroxysmal nocturnal hemoglobinuria (PNH). (oncologynurseadvisor.com)
- Children with very severe aplastic anemia have all of these same findings, in addition to a peripheral blood neutrophil count less than 0.2x 10 9 /L. (clinicalpainadvisor.com)
- Correction: Population pharmacokinetics of fludarabine in patients with aplastic anemia and Fanconi anemia undergoing allogeneic hematopoietic stem cell transplantation. (amedeo.com)
- Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic anaemia. (telomerescience.com)
- Although hematopoietic cells with a paroxysmal nocturnal hemoglobinuria (PNH) phenotype have been often detected in patients with aplastic anemia (AA), their pathologic significance remains unclear. (elsevier.com)
- Immunosuppressive therapy versus haploidentical transplantation in adults with acquired severe aplastic anemia. (amedeo.com)
- Aplastic anaemia can also lead to paroxysmal nocturnal haemoglobinuria (PNH), and vice versa, as in our experience some patients with PNH may later develop aplastic anaemia. (theaat.org.uk)
- 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)
- His main achievements concerned the Hemolytic anemias, a field in which he was a world leader. (wikipedia.org)