Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis.
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.
Behrens
Edward M
EM
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.
Kreiger
Portia A
PA
Cherian
Sindhu
S
Cron
Randy Q
RQ
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D015434:Panniculitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors*
D000305:Adrenal Cortex Hormones; D053590:Interleukin 1 Receptor Antagonist Protein; D017472:Receptors, Interleukin-1; D016572:Cyclosporine