Interleukin 1 Receptor Antagonist to Treat Cytophagic Histiocytic Panniculitis with Secondary Hemophagocytic Lymphohistiocytosis

Overview

Journal J Rheumatol

Specialty Rheumatology

Date 2006 Oct 3

PMID 17014024

Citations 29

Authors

Edward M Behrens

Portia A Kreiger

Sindhu Cherian

Randy Q Cron

Affiliations

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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.

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