Acute promyelocytic leukemia with JAK2 V617F and severe differentiation syndrome

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6 Scopus citations


Myeloproliferative neoplasms transformed into AML usually have a poor prognosis. We report a case of essential thrombocythemia with myelofibrosis that transformed into acute promyelocytic leukemia (APL) with both the t(15;17) translocation as well as the JAK2 V617F mutation. Clinically, this case was notable for severe differentiation syndrome despite treatment with high-dose dexamethasone. Cytokine production by differentiating APL cells was not directly abrogated by JAK2 inhibitors in vitro, suggesting that JAK2 V617F enhances the hyperinflammatory response downstream of cytokines. JAK1/2 inhibitors may therefore dampen the inflammatory cascade downstream of cytokine production, similar to glucocorticoids, and have a role in treating severe differentiation syndrome.

Original languageEnglish (US)
Pages (from-to)8-11
Number of pages4
JournalLeukemia Research Reports
Issue number1
StatePublished - Jan 1 2015


  • Acute promyelocytic leukemia (APL)
  • Differentiation syndrome
  • Essential thrombocythemia (ET)
  • JAK2
  • Myeloproliferative neoplasm (MPN)

ASJC Scopus subject areas

  • Hematology
  • Oncology


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