A contemporary approach to the diagnosis and management of polycythemia vera

The natural history of polycythemia vera (PV) includes an increased lifetime risk of thrombohemorrhagic complications and disease transformation into myelofibrosis with myeloid metaplasia and acute myeloid leukemia. The latter is the primary reason for the shortening of survival that becomes significant after the first decade of disease. Historic nonrandomized studies have suggested that aggressive phlebotomy improves survival in PV. However, prospective randomized studies have failed to demonstrate a better treatment than phlebotomy alone, in terms of survival. However, the addition of cytoreductive therapy to phlebotomy in high-risk patients with PV may reduce the risk of recurrent thrombosis. Other disease features of PV include aquagenic pruritus and microvascular disturbances such as erythromelalgia. This review outlines a practical approach to diagnosis, in addition to treatment of life-threatening and non-life-threatening complications of PV.