Essential thrombocytosis
From Biocrawler, the free encyclopedia.
Essential thrombocytosis (ET, essential thrombocythemia) is a rare and chronic blood disorder characterized by the overproduction of platelets (thrombocytosis). In some cases the disorder may be progressive, and (very rarely) evolves into acute leukemia or myelofibrosis.
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Epidemiology
A large portion of individuals with this disorder are asymptomatic (some studies suggest as many as two-thirds), which makes it difficult to estimate its true prevalence in the population. ET is diagnosed at a rate of about 2 to 3 per 100,000 individuals. It usually affects middle aged to elderly individuals (although it can affect children and young adults).
Cause
It is one of four rare myeloproliferative diseases in which an overproduction of blood platelet precursor cells (megakaryocytic hyperplasia) leads to an overproduction in blood platelets (peripheral thrombocytosis).
Symptoms
Aside from the overproduction of platelets, symptoms of ET include an enlarged spleen (splenomegaly), epistaxis (nosebleeds) and bleeding from gums and gastrointestinal tract. Thrombosis is more common.
Treatment
In cases where patients have life-threatening complications, the platelet count can be reduced rapidly through platelet apheresis (a procedure that removes platelets from the blood directly). Long-term decreases in platelet counts can reduce bleeding and clotting complications. Common medications include hydroxyurea, interferon-alpha, or anagrelide. Aspirin may also help decrease clotting.
External links
- Thrombocytosis, Essential (http://http://www.emedicine.com/med/topic2266.htm). Retrieved June 6, 2005.
