PT  - JOURNAL ARTICLE
AU  - Edward M Behrens
AU  - Portia A Kreiger
AU  - Sindhu Cherian
AU  - Randy Q Cron
TI  - Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis.
DP  - 2006 Oct 01
TA  - The Journal of Rheumatology
PG  - 2081--2084
VI  - 33
IP  - 10
4099  - http://www.jrheum.org/content/33/10/2081.short
4100  - http://www.jrheum.org/content/33/10/2081.full
SO  - J Rheumatol2006 Oct 01; 33
AB  - 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.