TY - CHAP M1 - Book, Section TI - Essential Thrombocythemia A1 - Beer, Philip A. A1 - Green, Anthony R. A2 - Kaushansky, Kenneth A2 - Lichtman, Marshall A. A2 - Prchal, Josef T. A2 - Levi, Marcel M. A2 - Press, Oliver W. A2 - Burns, Linda J. A2 - Caligiuri, Michael PY - 2015 T2 - Williams Hematology, 9e AB - SUMMARYEssential thrombocythemia is a clonal stem cell disorder characterized by an overproduction of platelets and associated with mutations in the JAK2, CALR, or MPL gene. Complications include thrombosis (predominantly arterial), hemorrhage, and progression to myelofibrosis or acute myeloid leukemia. Diagnosis requires exclusion of reactive thrombocytosis and other myeloid malignancies associated with a raised platelet count. Therapy is aimed at reducing thrombotic complications and includes modification of known cardiovascular risk factors and antiplatelet therapy for the majority of patients. Those at high risk of thrombosis are also considered for cytoreductive therapy with agents such as hydroxyurea, anagrelide or interferon-α. Although the majority of patients can expect to live for many years, mortality rates are increased compared to the general population as a consequence of disease complications. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1121098245 ER -