Polycythemia vera

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Primary polycythemia; Polycythemia rubra vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez's disease; Osler's disease; Polycythemia with chronic cyanosis - Myelopathic polycythemia; Erythrocytosis megalosplenica; Cryptogenic polycythemia

The objective of treatment is to reduce the high blood viscosity (thickness of the blood) due to the increased red blood cell mass and to prevent hemorrhage and thrombosis.

Phlebotomy is one method used to reduce the high blood viscosity. In phlebotomy, 1 unit (pint) of blood is removed weekly until the hematocrit is less than 45, then phlebotomy is continued as necessary.

Occasionally, chemotherapy may be given to suppress the bone marrow. Other agents such as interferon may be given to lower blood counts. Anegrelide may be given to lower platelet counts.

The use of anti-platelet therapy (such as aspirin) is controversial because it may cause stomach bleeding.

Allopurinol is given for hyperuricemia (gout).

Polycythemia vera usually develops slowly, and most patients treated appropriately do not experience any problems related to the disease. However, the abnormal bone marrow cells may begin to grow uncontrollably leading to acute myelogenous leukemia. This occurs in a minority of cases.

Patients with polycythemia vera also have an increased tendency to form blood clots that can result in strokes or heart attacks. Some patients may experience abnormal bleeding because their platelets are abnormal.

• Thrombosis (a cause of stroke or heart attack)
• Peptic ulcer disease
• Gastric bleeding
• Gout
• Leukemia
• Heart failure
• Myelofibrosis

Call your health care provider if symptoms of polycythemia vera develop.

Review Date: 10/31/2005
Reviewed By: William Matsui, M.D., Assistant Professor of Oncology, Division of Hematologic Malignancies, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD. Review provided by VeriMed Healthcare Network.