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

Edward M Behrens; Portia A Kreiger; Sindhu Cherian; Randy Q Cron

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

J Rheumatol. 2006 Oct;33(10):2081-4.

Authors

Edward M Behrens¹, Portia A Kreiger, Sindhu Cherian, Randy Q Cron

Affiliation

¹ Department of Pathology and Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4318, USA.

PMID: 17014024

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.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adrenal Cortex Hormones / therapeutic use
Cyclosporine / therapeutic use
Dose-Response Relationship, Drug
Drug Therapy, Combination
Female
Humans
Interleukin 1 Receptor Antagonist Protein / therapeutic use*
Lymphohistiocytosis, Hemophagocytic / diagnosis
Lymphohistiocytosis, Hemophagocytic / etiology*
Lymphohistiocytosis, Hemophagocytic / pathology
Panniculitis / complications*
Panniculitis / diagnosis
Panniculitis / drug therapy*
Panniculitis / pathology
Receptors, Interleukin-1 / antagonists & inhibitors*

Substances

Adrenal Cortex Hormones
Interleukin 1 Receptor Antagonist Protein
Receptors, Interleukin-1
Cyclosporine