The disease is caused by overgrowth of a blood cell precursor, and is a type of slowly-progressive, or chronic, malignancy. 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.
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