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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
Polycythemia vera is a disorder of the bone marrow. It mainly causes too many red blood cells to be produced. The numbers of white blood cells and platelets may increase as well..
This is a rare disorder that occurs more often in men than in women. It is not often seen in people under age 40. The problem is often linked to a gene defect called JAK2V617F. The cause of this gene defect is unknown.
Other symptoms that may occur with this disease:
The health care provider will perform a physical exam. You may also have the following tests:
This disease may also affect the results of the following tests:
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 unit of blood (about 1 pint) is removed each week until the number of red blood cells drops. The treatment is continued as needed.
Other treatments may include:
Taking aspirin to reduce the risk of blood clots may be an option for some people. However, aspirin increase the risk of stomach bleeding.
Ultraviolet-B light therapy can reduce the severe itching some patients experience.
The disease usually develops slowly. Most patients do not have problems related to the disease after being diagnosed. The condition is often diagnosed before severe symptoms occur.
Call your health care provider if symptoms of polycythemia vera develop.
Kremyanskaya M, Najfeld V, Mascarenhas J et al. The polycythemias. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 67.
Tefferi A. Polycythemias. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 169.
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