Polycythemia Vera: Is It Time to Rethink Treatment?

Introduction Polycythemia vera (PV) is the most frequent among the Philadelphia-negative myeloproliferative neoplasms (MPNs).1 PV is characterized by excessive myeloid cells production secondary to mutations in the Janus kinase 2 (JAK2) gene.2,3 The incidence rate is higher in the sixth decade of life and among males.4 Clinical phenotype is dominated by systemic symptoms, pruritus, and microvascular disturbances; splenomegaly may be detected in 30% of cases.5 In the long term, what impacts outcomes is the increased risk of thrombosis and evolution into post-PV myelofibrosis (PPV-MF) or blast phase (BP).6