TY - JOUR T1 - Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis. JF - The Journal of Rheumatology JO - J Rheumatol SP - 2081 LP - 2084 VL - 33 IS - 10 AU - Edward M Behrens AU - Portia A Kreiger AU - Sindhu Cherian AU - Randy Q Cron Y1 - 2006/10/01 UR - http://www.jrheum.org/content/33/10/2081.abstract N2 - 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. ER -