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Alternative namesEssential thrombocythemia; Essential thrombocytosis
DefinitionPrimary thrombocythemia is a condition of overproduction of platelets without a recognizable cause.
Causes, incidence, and risk factors
Primary thrombocythemia is caused by overgrowth of a blood cell precursor that progresses slowly. Although the platelets are primarily affected, the red blood cells and white blood cells are also involved.
The disease has similarities to polycythemia vera , chronic myelogenous leukemia , and myelofibrosis . Usually it affects people in middle age. Bleeding can occur from the gastrointestinal, respiratory , urinary tract, or skin.
The formation of blood clots , called thrombosis, may coincide with bleeding episodes. It may even cause strokes in some people. Risk factors are unknown. The incidence is about 3 out of 100,000 people.
Signs and tests
If a patient is having life-threatening complications, rapid decrease of the platelet count may be achieved through platelet pheresis, a procedure to remove platelets from the blood directly.
Long-term decrease of the platelet count using medications can reduce both bleeding and clotting complications. Most common medications include hydroxyurea, interferon-alpha, or anagrelide. For patients with a known clotting tendency, aspirin may help decrease clotting episodes.
Some patients do not need any treatment.
Expectations (prognosis)The outcome varies with reports ranging from prolonged periods without complications in some people, to fatalities from complications related to hemorrhage and thrombosis in others.
Calling your health care provider
Update Date: 8/18/2003Corey Cutler, M.D., M.P.H., F.R.C.P.C, Department of Medical Oncology, Dana-Farber Cancer Institute; Instructor in Medicine, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT