Thrombocytosis and essential thrombocythemia

摘要: Abstract Management of essential thrombocythemia (ET) starts with confirming the accuracy diagnosis and excluding congenital reactive thrombocytosis myeloid neoplasms which might mimic ET. Diagnosis acording to WHO criteria requires a platelet count ≥450×109/L, presence one three “driver” mutations, including JAK2, CALR, MPL or in their absence exclusion other causes thrombocytosis, bone marrow histology. Most patients ET have normal life expectancy low risk leukemic transformation fibrotic progression. Observation alone remains viable treatment option for “very low-risk” while all benefit from aspirin therapy, once- twice-daily schedule. In addition, cytoreductive is recommended thrombosis history, drugs choice this regard are hydroxyurea pegylated interferon-α. There currently no evidence support need JAK inhibitor therapy majority ET, regardless whether not they hydroxyurea-refractory