Current management strategies for polycythemia vera and essential thrombocythemia

Blood Rev. 2020 Jul:42:100714. doi: 10.1016/j.blre.2020.100714. Epub 2020 Jun 3.

Abstract

Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms characterized by increased rate of cardiovascular events, a varying burden of symptoms, and an intrinsic risk of evolution to secondary forms of myelofibrosis and acute leukemia; however, survival is only modestly reduced in most instances. In the last few years, following the description of driver mutations in JAK2, MPL and CALR, the diagnostic criteria for PV and ET were revised, making the identification of very early stages feasible. Scores for identifying patients at different risk of thrombosis were refined, and they largely guide therapeutic decisions. Treatment is therefore mainly focused on reduction of thrombosis risk, control of myeloproliferation, improvement of symptomatic burden, and management of disease-associated complications. New drugs recently entered the clinical arena, with the promise to improve overall patients' management. However, evidence of a disease-modifying potential is largely missing and represents a still unmet clinical need.

Keywords: Essential thrombocythemia; Hydroxyurea; JAK2; Polycythemia vera; Thrombosis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Disease Management
  • Humans
  • Polycythemia Vera / complications
  • Polycythemia Vera / diagnosis
  • Polycythemia Vera / therapy*
  • Thrombocythemia, Essential / complications
  • Thrombocythemia, Essential / diagnosis
  • Thrombocythemia, Essential / therapy*
  • Thrombosis / etiology
  • Thrombosis / prevention & control