How to Treat Essential Thrombocythemia and Polycythemia Vera

Abstract

Polycythemia vera and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms associated with thrombotic or hemorrhagic complications, and increased risk of transformation to myelofibrosis and acute myeloid leukemia. The main goal of therapy is aimed at preventing vascular events that are the leading cause of morbidity and mortality in these patients. Accordingly, risk stratification is the basis for deciding when to treat a patient with cytoreductive therapy. The European LeukemiaNet has developed a series of management recommendations for front-line and second-line therapy to provide the optimal treatment for the individual patient. There is still controversy about the efficacy and safety of several modalities of cytoreductive treatment in the long-term for both diseases and in the use of antiplatelet therapy in ET. The presence of JAK2V617F and CALR mutations in patients with ET has been related to different thrombotic risks, and this will probably lead to different therapeutic approaches in the near future. On the other hand, the near normal life expectancy of these patients makes a careful analysis of the benefits and risks associated with treatment essential. This review provides our current management strategy of patients with polycythemia vera and ET.

Keywords: Anagrelide; Antiplatelet therapy; Essential thrombocythemia; Hydroxyurea; Interferon; Polycythemia vera; Ruxolitinib.