Alternative names: ET, Essential Thrombocytosis, Essential Thrombocythaemia, Primary Thrombocytosis
Essential thrombocythemia is a rare chronic blood disorder characterized by the overproduction of platelets in the bone marrow. It is a slowly-progressing disorder with long symptom-free periods punctuated by thrombotic or hemorrhagic events.
The incidence of ET is between 6 and 25 cases per million people per year. People older than 60 are most likely to develop the condition, though 20% of those with this condition are under 40. The median age at onset is 65-70. Women are 1.5 times more likely than men to develop the condition.
There is evidence that exposure to chemicals or to electrical wiring may increase a person's risk for the condition.
Most ET patents show no symptoms; the condition is usually discovered during routine blood work. Where symptoms are present, they may include:
Not all those affected will require aggressive treatment. Based on their age, medical history, blood counts and lifestyles, patients are classed as either low or high risk for bleeding/blood clotting. Those with low risk are usually treated with low-dose aspirin (for headache and burning pain in the skin); those at high risk are given hydroxycarbamide and/or other treatments that reduce platelet count such as Hydroxyurea (Droxia, Hydrea), interferon-a and anagrelide (Agrylin), as well as aminocaproic acid to reduce bleeding.
Well-documented medical regimens can reduce and control the number of platelets, which reduces the risk of thrombotic or hemorrhagic events. A well-controlled ET patient can have a normal lifespan.
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