IL-1RA Agonist (Anakinra) in the Treatment of Multifocal Castleman Disease
- 1 December 2008
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Journal of Pediatric Hematology/Oncology
- Vol. 30 (12), 920-924
- https://doi.org/10.1097/mph.0b013e31818ab31f
Abstract
Multicentric Castleman disease (MCD), or multicentric angiofollicular lymph node hyperplasia, is an idiopathic lymphoproliferative disorder that is only seldom reported in children. The clinical and laboratory findings that characterize this peculiar disorder are thought to result from increased interleukin (IL)-6 production. We report herein the case of a 13-year-old boy with a long history of hectic fevers and abdominal pain, accompanied by stunted growth and elevated biologic markers of inflammation. Surgical biopsies of a pancreatico-splenic mass and of mesenteric lymph nodes revealed mixed-type MCD, which was diagnosed 6 years after the first clinical symptoms appeared. He received combination chemotherapy (cyclophosphamide, vinblastine) associated with a monoclonal B-cell antibody (Rituximab). This treatment was well tolerated but ineffective. Given the reported success of IL-1 blocking agents for treating Still disease, another IL-6 linked disorder, we attempted to treat him with anakinra, an IL-1RA agonist. His overall state normalized and both his clinical and biologic signs dramatically improved. This is the first report of anakinra treatment for MCD. We conclude that anti-IL-1 blocking agents could be an interesting treatment alternative for MCD, a chronic debilitating disease, which still carries a poor prognosis.Keywords
This publication has 19 references indexed in Scilit:
- Castleman diseaseCurrent Opinion in Hematology, 2007
- Multifocal Castleman Disease in PediatricsJournal of Pediatric Hematology/Oncology, 2005
- Rituximab therapy for HIV-associated Castleman diseaseBlood, 2003
- ANTI-INTERLEUKIN 6 (IL-6) RECEPTOR ANTIBODY SUPPRESSES CASTLEMAN'S DISEASE LIKE SYMPTOMS EMERGED IN IL-6 TRANSGENIC MICECytokine, 2002
- Y a-t-il une place pour l'interféron-α dans la stratégie thérapeutique de la maladie de Castleman multicentrique?La Revue de Médecine Interne, 2000
- Paediatric Castleman disease: report of seven cases and review of the literatureEuropean Journal of Pediatrics, 1999
- La maladie de CastlemanLa Revue de Médecine Interne, 1996
- Increased interleukin-6 (IL-6) production in a young child with clinical and pathologic features of multicentric Castleman's diseaseJournal of Clinical Immunology, 1994
- Expression of interleukin-6 in Castleman's diseaseHuman Pathology, 1993
- Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman's diseaseBlood, 1989