Polycythemia vera-associated complications: Pathogenesis, clinical manifestations, and effects on outcomes

Abstract

Polycythemia vera is a Philadelphia-negative chronic myeloproliferative neo-plasm, characterized by erythrocytosis, which is unique, compared to essential thrombocy-tosis and primary myelofibrosis. Though longevity can usually be expected, vascular morbidity is associated with this condition, as well as a propensity to evolve into myelofi-brosis (post-PV MF) and acute myeloid leukemia. In addition, patients can have a pro-nounced symptom burden. Herein, contributors to the symptomatic burden, as well as the thrombotic and transformative tendencies are reviewed. From a symptom perspective, some are explained by cytokine release, others by microvascular complications, whereas certain symptoms can herald disease evolution. Thrombosis has multifactorial contributors, including but not limited to gender, and inflammatory stress; investigators have recently hypothe-sized that microparticles and Neutrophil Extracellular Trap Formations may add to thrombotic burden. Finally, we examine the progression to post-PV MF as well as leukemic transformation, highlighting well-established risk factors including age and leukocytosis, certain treatments, and the presence of “non-driver” mutations.