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 ?
Impact of Jak2v617f mutational status on phenotypic features in essential thrombocythemia and primary myelofibrosis.
The report provides a snapshot of the global therapeutic landscape of Essential Thrombocythemia
Essential thrombocythemia, although rare, is an ominous comorbidity for coronary artery bypass surgery with an unpredictable prognosis.
Hino H, Teraski T, Hashimoto Y, Hara Y, Uchino M Cerebral infarction associated with mobile thoracic ascending aortic thrombus in a patient with essential thrombocythemia.
The World Health Organization (WHO) diagnostic criteria for PV, essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF) were revised after the discovery of JAK2 mutations in 2008 (for example: JAK2 V617F and JAK2 exon 12 mutations in virtually all patients with PV.
Epidemiology of the myeloproliferative disorders: essential thrombocythemia, polycythaemia vera and idiopathic myelofibrosis.
The 1950 hypothesis of Dameshek was confirmed by Vainchenker in France by the discovery in 2005 of the acquired JAK2V617F mutation as the cause of 3 phenotypes of classical inyeloproliferative neoplasia: essential thrombocythemia.
Myeloproliferative neoplasms (MPNs) (6) are a group of hematologic malignancies that include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF).
Thrombosis-free survival and life expectancy in 187 consecutive patients with essential thrombocythemia.
A two day workshop on the use of PCR technology for early diagnosis, monitoring and management of Beta Thalassemia and Mutations in JAK2 that have been implicated in polycythemia vera, essential thrombocythemia, myelofibrosis as well as other myeloproliferative disorders was conducted by joint effort of Departments of Biochemistry, Hematology and Molecular Pathology on 20th and 21st Feb.
Primary thrombocytosis (also called essential thrombocythemia, ET) is caused by autonomous production of platelets unregulated by the physiologic feedback mechanism to keep the count within the reference range.

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