ESSENTIAL THROMBOCYTHEMIA: UPDATE ON PATHOGENESIS, DIAGNOSIS, AND MANAGEMENT

Essential thrombocythemia (et), a member of the family of myeloproliferative neoplasms (mpn), is characterized by an overproduction of platelets and usually accompanied by thrombotic or hemorrhagic symptoms. jak2v617f mutation is present in about one-half of patients with et. mutations in mpl exon 10 are present in a further 4% of patients, with mpl w515l being the most common mutation. the diagnosis is made by exclusion of reactive thrombocytosis and other myeloid malignancies. bone marrow examination reveals an increase in megakaryocyte frequency with megakaryocyte clustering and nuclear hyperlobulation in the absence of significant reticulin fibrosis and significant increase in granulopoiesis and erythropoiesis. survival of patients with et had been similar to population controls during the first decade of the disease. yet, survival became significantly worse thereafter due to disease complications such as thrombosis and transformation to myelofibrosis or acute myeloid leukemia (aml). management paradigms have changed significantly over the last 20 years. this review aims to highlight the pathogenesis, diagnosis and current management in et