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

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 ?
Polycythemia Vera-PV 95% JAK2 V617F 1% JAK2 exon 12 4% other Essential Thrombocythemia-ET 50-60% JAK2 20-25% CALR 5-10% MPL 5-25% other Primary Myelofibrosis -PMF 50-60% JAK2 20-30% CALR 5-10% MPL 0-25% other ET: essential thrombocythemia; PMF:primary myelofibrosis; PV: policytemia vera Note: Table made from pie chart.
Front-line therapy in polycythemia vera and essential thrombocythemia. Blood Rev 2012; 26(5):205-211.
Efimova et al., "Changes in peripheral blood lymphocytes in polycythemia vera and essential thrombocythemia patients treated with pegylated-interferon alpha and correlation with [JAK2.sup.V617F] allelic burden," Experimental Hematology & Oncology, vol.
Wadenvik, "Diagnostic and differential criteria of essential thrombocythemia and reactive thrombocytosis," Leukemia & Lymphoma, vol.
(2011) Impact of leukocytosis on thrombotic risk and survival in 532 patients with essential thrombocythemia: a retrospective study.
Impact of Jak2v617f mutational status on phenotypic features in essential thrombocythemia and primary myelofibrosis.
A prognostic model to predict survival in 867World Health Organization-defined essential thrombocythemia at diagnosis: a study by the International Working Group on Myelofibrosis Research and Treatment.
Essential thrombocythemia (ET) is a clonal expansion of multipotential stem cells.
These present as essential thrombocythemia (ET) characterized by thrombocytosis, polycythemia vera (PV) with predominant erythrocytosis and concomitant leuko- and thrombocytosis, or myelofibrosis (MF) with expansion of megakaryocytes and reactive bone marrow fibrosis [2].
[7.] Tefferi A, Wassie EA, Guglielmelli P, et al Type 1 versus type 2 calreticulin mutations in essential thrombocythemia: A collaborative study of 1027 patients.
(5) Myeloid neoplasms with 3q21q26 anomalies are not only seen in acute myeloid leukemias (AMLs), (6,7) but also in myelodysplastic syndromes (MDS) (5); blast crisis of myeloproliferative neoplasm (MPN), such as chronic myelogenous leukemia (8) or leukemic transformation of essential thrombocythemia (9); and even in some MDS/myeloproliferative neoplasm cases, such as chronic myelomonocytic leukemia.
Global Markets Direct's, 'Essential Thrombocythemia - Pipeline Review, H1 2016', provides an overview of the Essential Thrombocythemia pipeline landscape.

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