About Essential Thrombocythemia

The myeloproliferative disease, essential thrombocythemia (ET), is a chronic blood disorder characterized by increased numbers of platelets in the blood. These platelets may have abnormal function, which can lead to an increased risk of thrombotic or hemorrhagic complications. Patients with ET may also develop myelofibrosis or acute myeloid leukemia.

Life expectancy may be normal in the majority of patients with ET in the first decade after diagnosis, but subsequent survival is reduced approximately two-fold compared with the general population. Currently used treatments, such as hydroxyurea and anagrelide, can be effective in reducing platelet counts in patients with ET, but do not alter the underlying biology of the disease, and clinical resistance or intolerance to these agents occurs in a proportion of patients. The utility of interferon-alpha, which can induce molecular responses in some patients, is limited by tolerability.

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