Recent advances in polycythemia vera treatment

Abstract

Since the discovery of the gain-of-function mutation JAK2 V617F, significant progress has been made in clarifying the pathology and developing novel agents for myeloproliferative neoplasms, including polycythemia vera (PV). The treatment strategy for PV is to first classify patients into either high- or low-risk groups for thrombosis. All patients with PV should be treated with low-dose aspirin and phlebotomy. In addition, for high-risk PV patients, cytoreductive therapy is recommended. Although hydroxyurea (HU) is the most popular agent for PV treatment, the advantages of ruxolitinib, a JAK inhibitor, for patients who are intolerant or resistant to HU were recently reported. Furthermore, the ability of interferon-α to selectively eliminate the malignant clone and induce complete molecular response was previously demonstrated. In this article, important clinical trials associated with the treatment strategy for PV and recent advances in PV treatments are described.