Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia

Michelle A. Elliott, A. Tefferi

Research output: Contribution to journalReview article

346 Scopus citations

Abstract

Despite decades of clinical and laboratory research, relatively little has been accomplished concerning the pathogenesis as well as the identification of risk factors for thrombosis and bleeding in myeloproliferative disorders. In polycythaemia vera, the pro-thrombotic effect of an elevated haematocrit is well established. In contrast, thrombocytosis per se has not been similarly incriminated in essential thrombocythaemia. In both conditions, advanced age and the presence of a prior event identify thrombosis-prone patients. There is increasing evidence to suggest an additional role by leucocytes that might partly explain the antithrombotic effects of myelosuppressive therapy. A substantial minority of affected patients display reduced levels of high molecular weight von Willebrand protein in the plasma during extreme thrombocytosis and it is believed that this might explain the bleeding diathesis of such patients. Recent controlled studies support the therapeutic value of hydroxyurea and aspirin in essential thrombocythaemia and polycythaemia vera, respectively. The current communication will address the incidence, phenotype, pathogenesis, risk factors, prevention, and treatment of both thrombosis and haemorrhage in these disorders.

Original languageEnglish (US)
Pages (from-to)275-290
Number of pages16
JournalBritish journal of haematology
Volume128
Issue number3
DOIs
StatePublished - Feb 1 2005

Keywords

  • Essential thrombocythaemia
  • Haemorrhage
  • Polycythaemia vera
  • Thrombosis

ASJC Scopus subject areas

  • Hematology

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