How I treat patients with low-risk polycythemia vera who require cytoreduction

Polycythemia vera (PV) was first described by Louis Henri Vaquez in 1892. It is a chronic hematological malignancy that affects both older and young patients. Perhaps because of lack of a curative treatment and the perceived toxicities of prior therapies, our focus in the past was to intensify treatment only for patients at higher risk of thrombosis. Recent triggers to challenge this approach include the following: a recognition that low-risk PV is not "no risk," our ability to better recognize patients who would benefit from more intensive therapy from the perspective of thrombosis, and data showing that some treatments may reduce risk of transformation to myelofibrosis. Furthermore, there is emergent evidence that molecular monitoring may identify an improvement in disease state translating to improved overall survival. Here, we describe clinical situations that would trigger the use of cytoreductive treatment for patients with low-risk PV as well as our approach to choosing a specific cytoreductive agent and how to effectively monitor treatment.