A clinical update in polycythemia vera and essential thrombocythemia

Am J Med. 2000 Aug 1;109(2):141-9. doi: 10.1016/s0002-9343(00)00449-6.


Polycythemia vera and essential thrombocythemia pose specific management issues that distinguish them from other chronic myeloproliferative disorders. They are associated with a better prognosis, as well as a variable risk of thrombohemorrhagic complications. In addition, essential thrombocythemia occurs comparatively more often in young people and women. Treatment strategies for patients with polycythemia vera and essential thrombocythemia must consider the possibility of long-term survival, morbidity from thrombotic complications, transformation into myelofibrosis with myeloid metaplasia or acute myeloid leukemia, and the effect of specific therapies on the incidence of leukemic transformation and on pregnancy. There is increasing concern about the possible leukemogenic effect of hydroxyurea. Newer therapeutic agents, including interferon alpha and anagrelide, are being used more often. Ongoing studies are reexamining the effects of low-dose aspirin in preventing thrombotic complications.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Age Factors
  • Carcinogens / adverse effects
  • Cell Transformation, Neoplastic / pathology
  • Chronic Disease
  • Enzyme Inhibitors / adverse effects
  • Female
  • Hemorrhage / etiology
  • Humans
  • Hydroxyurea / adverse effects
  • Leukemia / chemically induced
  • Myeloproliferative Disorders / classification
  • Polycythemia Vera / diagnosis
  • Polycythemia Vera / etiology
  • Polycythemia Vera / therapy*
  • Pregnancy
  • Pregnancy Complications, Hematologic
  • Prognosis
  • Risk Factors
  • Sex Factors
  • Survival Rate
  • Thrombocythemia, Essential / diagnosis
  • Thrombocythemia, Essential / etiology
  • Thrombocythemia, Essential / therapy*
  • Thrombosis / etiology


  • Carcinogens
  • Enzyme Inhibitors
  • Hydroxyurea