Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis

Academic Article

Abstract

  • Cytophagic histiocytic panniculitis (CHP) is a rare, usually fatal disease of childhood: it typically presents with daily high spiking fevers and severe panniculitis. Hemophagocytosis from macrophage activation during a cytokine storm can result in pancytopenia and disseminated intravascular coagulopathy. We describe a 14-year-old girl with long-standing CHP who developed severe hemophagocytic lymphohistiocytosis, which responded to treatment with a combination of high dose corticosteroids. cyclosporine, and the interleukin 1 receptor antagonist, anakinra.
  • Published In

    Pubmed Id

  • 27661769
  • Author List

  • Behrens EM; Kreiger PA; Cherian S; Cron RQ
  • Start Page

  • 2081
  • End Page

  • 2084
  • Volume

  • 33
  • Issue

  • 10