Essential thrombocythaemia

Last updated: Tuesday, 30, November, 2010
Key InformationAppropriate Tests

Essential thrombocythaemia is a myeloproliferative disorder in which the haemopoietic stem cell proliferation leads to a predominant increase in platelet numbers, usually with neutrophilia.

Clinical history to exclude reactive thrombocytosis or hyposplenism & PRV+MRD , FBC, blood film, marrow aspirate and trephine biopsy ± chromosomes, erythropoitin, Jak2 (V64F) indicated.

See also Myeloproliferative disorders

Features

Thrombocytosis ± giant platelets

Splenomegaly

Splenic infarction

Thrombosis (arterial and venous)

Bleeding, especially from

  • Platelet dysfunction 

Variable abnormalities of platelet function have been described.

The bleeding time does not predict spontaneous or surgical bleeding.

  • Acquired von Willebrand's disease
See von Willebrand's disease (VWD)-acquired

Disease progression or transformation

Uncommon ~5% in patients not exposed to alkylating agents.