Introduction: New treatment options for polycythemia vera (PV) have recently become available. This article reviews current treatment strategies for PV with a focus on anagrelide and ruxolitinib.
Areas covered: The current treatment of PV is directed towards reducing the risk of additional thrombotic events. In addition to phlebotomy and aspirin, patients who are believed to have high-risk disease also receive cytoreductive therapy. Hydroxyurea (HU) and PEG-IFN are most commonly used first-line treatments. The use of anagrelide in PV is limited to situations where patient develops thrombohemorrhagic episodes in the setting of extreme thrombocytosis. The role of ruxolitinib in treatment of PV has not been fully established but based on a recently completed Phase III trial it will likely serve as a second-line option for patients with systemic symptoms.
Expert opinion: HU and PEG-IFN are frontline therapies for patients with high-risk PV. Anagrelide use should be restricted to patients with clinical consequences of extreme thrombocytosis. Ruxolitinib is a treatment option for patients who fail frontline therapies. However, long-term effects and toxicities are not yet fully known.
Keywords: JAK inhibitor; JAK2V617F; anagrelide; hydroxyurea; myeloproliferative neoplasm; polycythemia vera; ruxolitinib.