title: Leukemic transformation in essential thrombocythemia.
abstract: Essential thrombocythemia patients develop acute myeloid leukemia (AML) at a rate of 1-4% during a median follow-up of 7-10 years. The risk increases with advanced age, anemia, platelet count ≥ 1000 × 10(9)/l, the presence of ≥ 2 somatic mutations and after the first decade of diagnosis. The use of alkylating agents and (32)radiophosphorus, particularly in higher doses, but not hydroxyurea and anagrelide, increases the risk. AML in essential thrombocythemia patients is frequently associated with unfavorable cytogenetics and poor prognosis. In young and fit patients, AML-type induction chemotherapy followed by allogeneic stem cell transplantation may offer the best chance of long-term disease control. In select elderly patients with poor performance status, hypomethylating agent such as azacytidine may prolong survival.
authors.forename: Vijaya Raj
authors.initials: VR
authors.lastname: Bhatt
mesh_terms: D001374:Azacitidine / Q000627:therapeutic use; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000473:pathology*; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D015470:Leukemia, Myeloid, Acute / Q000209:etiology / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D009154:Mutation; D011379:Prognosis; D012307:Risk Factors; D033581:Stem Cell Transplantation; D013920:Thrombocythemia, Essential / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D014184:Transplantation, Homologous
chemical_list: C507924:JAK2 protein, human; D053614:Janus Kinase 2; D001374:Azacitidine
keywords: CALR mutation; JAK2 V617F mutation; acute myeloid leukemia; allogeneic stem cell transplantation; cytoreductive drugs; essential thrombocythemia; induction chemotherapy