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Alternative NamesPrimary 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
Definition Return to top
Polycythemia vera is an abnormal increase in the number of blood cells (primarily red blood cells) produced by the bone marrow.
Causes Return to top
Polycythemia vera is a disorder of the bone marrow. It causes too much production of white blood cells, red blood cells, and platelets.
It is a rare disease that occurs more often in men than women, and is rare in patients under age 40. The exact cause is unknown.
Symptoms Return to top
Note: Symptoms are due to increased blood thickness and clotting.
Other symptoms that may occur with this disease:
Exams and Tests Return to top
The health care provider will perform a physical exam. Tests that may be done include:
This disease may also affect the results of the following tests:
Treatment Return to top
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.
The use of blood thinners (such as aspirin) is controversial because it may cause stomach bleeding. However, it does prevent blood clots.
Outlook (Prognosis) Return to top
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:
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.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if symptoms of polycythemia vera develop.
References Return to top
Tefferi A. Polycythemias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 172.Update Date: 3/2/2009 Updated 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.