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In hematology, essential thrombocythemia (ET) is a rare chronic blood cancer (myeloproliferative neoplasm) characterised by the overproduction of platelets (thrombocytes) by megakaryocytes in the bone marrow. [3] It may, albeit rarely, develop into acute myeloid leukemia or myelofibrosis. [3]
However, in essential thrombocythemia where platelet counts are over 750x10 9 /L or 1,000x10 9 /L, especially if there are other risk factors for thrombosis, treatment may be needed. Selective use of aspirin at low doses is thought to be protective.
Anagrelide (Agrylin/Xagrid, Shire and Thromboreductin, AOP Orphan Pharmaceuticals AG) is a drug used for the treatment of essential thrombocytosis (also known as essential thrombocythemia), or overproduction of blood platelets. It also has been used in the treatment of chronic myeloid leukemia. [2]
A third Imago-sponsored global clinical trial for the treatment of essential thrombocythemia was begun in 2020. [10] The results of this global study have also been presented at the American Society of Hematology and the European Hematology Association. A fourth trial begun in 2023 for the treatment of polycythemia vera is ongoing. [11]
Hydroxycarbamide, also known as hydroxyurea, is an antimetabolite medication used in sickle-cell disease, essential thrombocythemia, chronic myelogenous leukemia, polycythemia vera, and cervical cancer. [4] [5] In sickle-cell disease it increases fetal hemoglobin and decreases the number of attacks. [4] It is taken by mouth. [4]
Treatment is mainly directed towards the underlying condition. [2] [3] Other measures may include giving platelets, cryoprecipitate, or fresh frozen plasma. [2] Evidence to support these treatments, however, is poor. [2] Heparin may be useful in the slowly developing form. [2] About 1% of people admitted to hospital are affected by the ...
In the United States, fedratinib is indicated for the treatment of adults with intermediate-2 or high-risk primary or secondary (following polycythemia vera or essential thrombocythemia) myelofibrosis.
Essential thrombocythemia (ET) is a disorder characterized by elevated numbers of circulating platelets. The disease occurs in 1–2 per 100,000 people. The disease occurs in 1–2 per 100,000 people.