essential thrombocythemia


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thrombocythemia

 [throm″bo-si-the´me-ah]
essential thrombocythemia (hemorrhagic thrombocythemia) a clinical syndrome with repeated spontaneous hemorrhages, either external or into the tissues, and a greatly increased number of circulating platelets, considered one of the myeloproliferative disorders. Called also megakaryocytic leukemia.

essential thrombocythemia

A myeloproliferative neoplasm characterized by extreme thrombocytosis. Approximately 50% of essential thrombocythemia cases are characterized by presence of the JAK2 mutation. See thrombocytosis.

essential thrombocythemia

Essential thrombocytosis Hematology A primary myeloproliferative disorder of older–age 55-75–adults, or less commonly of young ♀, with a platelet count is > 600 x 109/L; ET has many clinical features of P vera; it affects the same age group, is accompanied by splenomegaly, has similar BM findings, intensity of leukocytosis Diagnosis Polycythemia Vera Study Group criteria Treatment Hydroxyurea, a myelosuppressant, prevents recurrent thromboses. See Polycythema vera.
Essential thrombocythemia-diagnostic criteria
1. Platelets > 1 x 109 /L (US: < 1000/mm3)
2. Hb < 2.05 mmol/L (US < 13.0 g/dL)
3. Iron in BM or if absent, little ↑ in Hb after 1 month of oral iron therapy
4. Absent marrow fibrosis by biopsy and
5. Absent Philadelphia chromosome
Polycythemia Vera Study Group
References in periodicals archive ?
Clinical profile of homozygous JAK2617V4F mutation in patients with polycythemia vera or essential thrombocythemia.
Clinical and laboratory features of platelet-mediated thrombosis and bleeding complications, and the molecular etiology of essential thrombocythemia and polycythemia vera: therapeutic implications.
Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis.
Essential thrombocythemia (ET) is a BCR-ABL1-negative myeloproliferative disorder characterized by stem cell-derived clonal myeloproliferation leading to thrombocytosis.
Essential thrombocythemia (ET) is a MPN that presents with an elevated platelet count.
Prevalence of polycythemia vera and essential thrombocythemia.
Recently MPL mutations (MPL W515L or MPL W515K) were described in patients with essential thrombocythemia (ET) and primary (idiopathic) myelofibrosis (PMF).
Identify the mutations associated with essential thrombocythemia (ET).
We studied SDF-1 gene polymorphisms in polycythemia vera (PV) and essential thrombocythemia (ET) to determine the frequency of the SDF1-3'A polymorphism and to evaluate its impact on susceptibility to these diseases and on the occurrence of thrombotic complications.
Objective: Myeloproliferative neoplasms (MPNs) like essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF) are acquired clonal hematopoietic stem cell disorders and originate from a multipotent hematopoietic stem cell.
The diagnosis of essential thrombocythemia requires a sustained platelet count:

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