Current management in polycythemia vera

Myeloproliferative diseases (MPD) include polycythemia vera (PV), essential thrombocythemia, agnogenic myeloid metaplasia, and chronic myelogenous leukemia. The focus of this report is on PV, which is characterized by an increase in red blood cells, granulocytes, and platelets. Complications associated with PV are an increased risk of thrombosis and abnormal bleeding. Phlebotomy to a hematocrit less than 45% is the mainstay of treatment for erythrocythemia, but may further increase the platelet count, necessitating the use of a platelet-lowering agent in conjunction with phlebotomy. Other treatment strategies include low-dose aspirin or other antithrombotic therapy and cytoreduction. Mounting evidence of the leukemogenicity and mutagenicity of radioactive phosphorus and alkylating agents, as administered using "conventional" regimens, has restricted the liberal use of these treatments. Three drugs have emerged as useful because of their efficacy in reducing the elevated platelet count: anagrelide, hydroxyurea (HU), and interferon alfa (IFN). It is clear that no single agent satisfies all the needs for cytoreduction that arise during the course of PV. Future protocols should be designed that draw on the large body of experience already gained with these drugs to transcend the limitations of single-agent therapy and to improve quality of life as well as survival. Semin Hematol 38(suppl 2):25-28.