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 goal of treatment is to reduce the thickness of the blood and prevent bleeding and clotting.

A method called phlebotomy is used to decrease blood thickness. One pint of blood is removed weekly until the hematocrit level is less than 45, then therapy is continued as needed.

Occasionally, chemotherapy (specifically hydroxyurea) may be given to suppress the bone marrow. Interferon may also be given in an attempt to lower blood counts. A medicine called anegrelide may be given to lower platelet counts.

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The use of blood thinners (such as aspirin) is controversial because it may cause stomach bleeding. However, it does prevent blood clots.

The disease usually develops slowly. Most patients do not experience any problems related to the disease after being diagnosed.

The following complications occur in a small number of patients:

• The abnormal bone marrow cells may begin to grow uncontrollably in some patients, leading to the development of acute myelogenous leukemia (AML).
• The bone marrow may develop a scarring condition called myelofibrosis.This condition may lead to dangerously low levels of white blood cells, red blood cells, and platelets.

Patients with polycythemia vera are also more likely to form blood clots that can cause strokes or heart attacks. Some patients may experience abnormal bleeding because their platelets are abnormal.

• Bleeding from the stomach or other parts of the intestinal tract
• Gout
• Heart failure
• Leukemia
• Myelofibrosis
• Peptic ulcer disease
• Thrombosis (blood clotting, which can cause stroke or heart attack)

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

Review Date: 03/02/2009
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org).