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Alternative names Return to top
Essential thrombocythemia; Essential thrombocytosisDefinition Return to top
Primary thrombocythemia is a condition of overproduction of platelets without a recognizable cause.Causes, incidence, and risk factors Return to top
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.
Symptoms Return to top
Signs and tests Return to top
Treatment Return to top
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) Return to top
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.Complications Return to top
Calling your health care provider Return to top
Update Date: 8/18/2003 Updated by: Corey 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.
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Page last updated: 28 October 2004 |