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Essential Thrombocythemia

  • Ayalew Tefferi
Part of the Hematologic Malignancies book series (HEMATOLOGIC)

Abstract

Essential thrombocythemia (ET) was first described in 1934, classified as a myeloproliferative disorder (MPD) in 1951, and accepted as a distinct clinicopathologic entity in 1960. Formal diagnostic criteria were established in the 1970s and in 1981; ET was recognized as a clonal stem cell disorder. In 2005, an activating JAK2 mutation (Jak2V617F) was demonstrated in a spectrum of MPDs, including ET where the mutational frequency is estimated at 50%. In ET, Jak2V617F has been associated with higher levels of hemoglobin and leukocyte count, higher risk of transformation into polycythemia vera (PV), and advanced age at diagnosis. In contrast, it is currently not clear if the specific mutation has an effect on survival, thrombosis risk, or leukemic transformation rate. Life expectancy in ET is significantly shorter than the control population but the difference may not be apparent in the first 10 years. The 20-year risk of both leukemic and fibrotic transformation is less than 10%. Clinical course is usually indolent with a minority of the patients experiencing thrombohemorrhagic complications. There is increasing evidence regarding the thrombogenic role of neutrophils in ET and this might partly explain the superior overall performance by hydroxyurea, compared to anagrelide, in a recent randomized study.

Keywords

Polycythemia Vera Essential Thrombocythemia Myeloproliferative Disorder Systemic Mastocytosis Jak2 V617F 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Berlin Heidelberg 2007

Authors and Affiliations

  • Ayalew Tefferi
    • 1
  1. 1.Division of HematologyMayo ClinicRochesterUSA

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