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 ?
Characterization of 35 new cases with four different MPL W515 mutations and essential thrombocytosis or primary myelofibrosis.
Characterization of 35 new cases with four different MPLW515 mutations and essential thrombocytosis or primary myelofibrosis.
It is well established that essential thrombocytosis in myeloproliferative diseases is associated with an increased risk of thrombosis due to the presence of giant platelets (1).
Unlike essential thrombocytosis, which can cause both arterial and venous thrombosis (12), reactive thrombocytosis has been traditionally considered as inconsequential without requiring anti-platelet therapy (1,3).
One case (Case 10) was a female at 34 weeks gestation with a previous history of Essential Thrombocytosis and thrombosis, referred for investigation because the haematologist had concerns regarding delivery, and this case was found to have normal platelet function plus normal (albeit high levels of) von Willebrand factor.
VWD 10 Previous Essential Thrombocytosis and previous normal portal vein thrombosis post splenectomy; now 34 wks gestation; current normal platelet count 11 Unexplained serious bleeding (neural equivocal haematoma); previous abnormal platelet function while on medication Case VWD LTA LTA Significant No.
platelet count >1000 K/[micro]L [15]), reactive thrombocytosis is a more common cause of thrombocytosis than is primary or essential thrombocytosis (1).
Essential thrombocytosis is the increased production of platelets in the absence of other myeloproliferative disorders.
Essential thrombocytosis is not a well-recognized cause for arterial and venous thrombosis (20); however, case reports of patients with arterial and venous thrombosis requiring intervention have been reported in the literature (21, 22).
Essential thrombocytosis is a diagnosis of exclusion by ruling out known causes of reactive thrombocytosis.
This patient is a delightful 56-year-old woman who came to the clinic with a diagnosis of essential thrombocytosis, a blood disease related to leukemia.

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