• M Jouvray, L Terriou, V Meignin, B Bouchindhomme, M Jourdain, M Lambert, G Lefevre, E Hachulla, P-Y Hatron, L Galicier, and D Launay.
• Faculté de médecine, université Lille Nord de France, 59037 Lille, France; Service de médecine interne, Centre national de référence de la sclérodermie systémique, hôpital Claude-Huriez, CHRU de Lille, rue Michel-Polonovski, 59037 Lille cedex, France.
• Rev Med Interne. 2016 Jan 1; 37 (1): 53-7.

IntroductionMulticentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia.Case ReportWe report a 32-year-old woman with an association of oligoanuria, anasarca, thrombotic microangiopathy with features compatible with adult-onset Still disease. The outcome was initially favorable with corticosteroids, immunoglobulins and plasmapheresis but with the persistence of relapses marked by severe autonomic syndrome and necessity of high dose corticosteroids. The diagnosis of mixed type Castleman's disease, HHV8 and HIV negative, was obtained four years after the onset of symptoms by a lymph node biopsy. The outcome was favorable after tocilizumab and corticosteroids but tocilizumab had to be switched to anakinra to ensure a proper and long-lasting control of the disease.ConclusionOur patient partially fits the description of TAFRO syndrome (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly), a MCM rare variant, recently described in Japanese patients.Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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