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The three major myeloproliferative syndromes are polycythemia vera, idiopathic myelofibrosis, and essential thrombocytosis. All are clonal disorders of hematopoietic stem cells and all are associated with a mutation in the JAK2 kinase (V617F) that results in activation of the kinase. The mutation is seen in 90% of pts with polycythemia vera and ~45% of pts with idiopathic myelofibrosis and essential thrombocytosis.