 |
|
|
 |
Case Report
Interleukin 1 Receptor Antagonist to Treat Cytophagic Histiocytic Panniculitis with Secondary Hemophagocytic Lymphohistiocytosis
EDWARD M. BEHRENS, PORTIA A. KREIGER, SINDHU CHERIAN, and RANDY Q. CRON 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. (J Rheumatol 2006;33:2081-4) Key Indexing Terms:
PANNICULITIS From the Department of Pathology and Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA, USA. Dr. Behrens was funded by an Amgen scholarship. Dr. Cron was supported in part by grants from the Nickolett Family Awards Program for JRA Research and the Ethel Brown Foerderer Fund for Excellence. E.M. Behrens, MD, Fellow Physician, Department of Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia; P.A. Kreiger, MD, Fellow Physician, Department of Pathology, Children's Hospital of Philadelphia; S. Cherian, MD, Fellow Physician, Department of Pathology, Hospital of the University of Pennsylvania; R.Q. Cron, MD, PhD, Assistant Professor, Department of Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia. Address reprint requests to Dr. R.Q. Cron, Children's Hospital of Philadelphia, 3615 Civic Center Blvd., ARC 1102B, Philadelphia, PA 19104-4318, USA. E-mail: rqcron@mail.med.upenn.edu Accepted for publication May 26, 2006.
|
