Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis
Behrens, E.M.; Kreiger, P.A.; Cherian, S.; Cron, R.Q.
Journal of Rheumatology 33(10): 2081-2084
2006
ISSN/ISBN: 0315-162X PMID: 17014024 Document Number: 602895
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 hemophagoeytic lymphohistiocytosis, which responded to treatment with a combination of high dose corticosteroids, cyclosporine, and the interleukin 1 receptor antagonist, anakinra.