Essential thrombocythaemia: a phenotypic spectrum with polycythaemia vera – a case report

Abstract

Essential thrombocythaemia (ET) is a non-leukaemic myeloproliferative neoplasm (MPN) associated marked proliferation of the megakaryocytes and elevated platelet count (≥450,000/mm³). This condition is characterized by splenomegaly, vascular complications, thrombotic and / or hemorrhagic clinical course episode. ET despite being a distinct clinical entity presents with characteristic phenotypic spectrum similar to the other non-leukemic MPNs including polycythaemia vera (PV) and primary myelofibrosis (PMF) and are characterized by the involvement of similar genetic drivers. In this report, we present a case of essential thrombocythaemia in a woman previously diagnosed with uterine fibroid and an intractable thrombotic complication. We also highlight the relevance of therapeutic phlebotomy as an important approach for management of thrombotic events.

Keywords: Essential thrombocythaemia, Polycythaemia vera, myeloproliferative neoplasm, thrombosis, platelet count