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Prognosis and treatment of polycythemia vera

Author
Ayalew Tefferi, MD
Section Editor
Stanley L Schrier, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Polycythemia vera (PV) is one of the myeloproliferative neoplasms (MPN) (table 1), a group of hematopoietic stem cell-derived malignancies that are characterized by clonal proliferation of myeloid cells with variable degrees of morphologic maturity. PV is distinguished from other MPNs by the presence of an elevated red blood cell mass (ie, erythrocytosis), and is associated with an increased risk for thromboembolic events, leukemic transformation, and/or myelofibrosis. (See "Overview of the myeloproliferative neoplasms".)

This topic will review the prognosis and treatment of PV. Approaches to the patient with suspected PV or polycythemia in general are discussed separately. (See "Clinical manifestations and diagnosis of polycythemia vera" and "Diagnostic approach to the patient with polycythemia".)

GOALS OF CARE

Survival of patients with PV who receive contemporary treatment is typically decades-long, but symptoms (eg, pruritus, erythromelalgia, splenomegaly), complications (eg, venous or arterial thrombotic events), and hematologic transformation (eg, myelofibrosis, acute myeloid leukemia, myelodysplastic syndromes) cause significant morbidity and limit life expectancy. (See 'Prognosis' below.)

While not curative, modern therapy for PV can relieve symptoms and prolong survival. The goals of care are to reduce the risk of first and/or recurrent thrombosis, prevent bleeding events, ameliorate the symptom burden, and minimize the risk of evolution to post-PV myelofibrosis (MF) and acute myeloid leukemia/myelodysplastic syndrome (AML/MDS) [1].

While no drug has been shown to lower the risk of hematologic transformation to MF or AML/MDS, current treatment approaches generally avoid agents known to increase this risk.

                                  
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Literature review current through: Sep 2017. | This topic last updated: Sep 07, 2017.
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