Leukemic transformation in essential thrombocythemia

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10 Scopus citations

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 × 109/l, the presence of ≥2 somatic mutations and after the first decade of diagnosis. The use of alkylating agents and 32radiophosphorus, 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.

Original languageEnglish (US)
Pages (from-to)2593-2602
Number of pages10
JournalFuture Oncology
Volume10
Issue number16
DOIs
StatePublished - Dec 1 2014

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Keywords

  • CALR mutation
  • JAK2 <sup>V617F</sup> mutation
  • acute myeloid leukemia
  • allogeneic stem cell transplantation
  • cytoreductive drugs
  • essential thrombocythemia
  • induction chemotherapy

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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