Abstract

Review Article

Essential thrombocythemia: Biology, clinical features, thrombotic risk, therapeutic options and outcome

Vincenzo Accurso*, Santoro M, Contrino AD, Casimiro P, Raso S, Carlisi M, Sardo M, Perez A, Di Piazza F, Russo A and Siragusa S

Published: 02 September, 2019 | Volume 3 - Issue 1 | Pages: 053-059

Essential Thrombocythemia (ET) is currently classified as a Philadelphia negative myeloproliferative neoplasm (MPN) together with polycythemia vera (PV) and primary myelofibrosis (PMF); the latter can be further divided in pre-fibrotic primary myelofibrosis (pre-PMF) and overt myelofibrosis, as listed in the revised 2016 World Health Organization classification of myeloid malignancies (WHO 2016). Overall, respect to the others MPNs, ET is characterized by favorable prognosis, lower life expectancy if compared to the control population, increased risk of thrombohemorrhagic complications along with possible evolution in myelofibrosis and leukemic transformation. In this review the authors will review current knowledge on biology, clinical aspects, prognosis and stratification of thrombotic risk, therapeutic options and outcome in ET patients.

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