title: [Pseudo-adult Still's disease, anasarca, thrombotic thrombocytopenic purpura and dysautonomia: An atypical presentation of multicentric Castleman's disease. Discussion of TAFRO syndrome].
abstract: BACKGROUND
  Multicentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia.
  
  
  METHODS
  We 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.
  
  
  CONCLUSIONS
  Our patient partially fits the description of TAFRO syndrome (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly), a MCM rare variant, recently described in Japanese patients.
authors.affiliation: 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.
authors.forename: M
authors.initials: M
authors.lastname: Jouvray
authors.affiliation: 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.
authors.forename: L
authors.initials: L
authors.lastname: Terriou
authors.affiliation: Anatomie et cytologie pathologique, hôpital Saint-Louis, 75010 Paris, France.
authors.forename: V
authors.initials: V
authors.lastname: Meignin
authors.affiliation: Institut de pathologie, centre de biologie pathologie, CHRU de Lille, 59037 Lille, France.
authors.forename: B
authors.initials: B
authors.lastname: Bouchindhomme
authors.affiliation: Service de réanimation polyvalente, hôpital Roger-Salengro, CHRU de Lille, 59037 Lille, France.
authors.forename: M
authors.initials: M
authors.lastname: Jourdain
authors.affiliation: 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.
authors.forename: M
authors.initials: M
authors.lastname: Lambert
authors.affiliation: 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; EA2686, 59037 Lille, France.
authors.forename: G
authors.initials: G
authors.lastname: Lefevre
authors.affiliation: 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; EA2686, 59037 Lille, France.
authors.forename: E
authors.initials: E
authors.lastname: Hachulla
authors.affiliation: 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.
authors.forename: P-Y
authors.initials: PY
authors.lastname: Hatron
authors.affiliation: Service de médecine interne, hôpital Saint-Louis, 75010 Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Galicier
authors.affiliation: 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; EA2686, 59037 Lille, France. Electronic address: david.launay@chru-lille.fr.
authors.forename: D
authors.initials: D
authors.lastname: Launay
mesh_terms: D000328:Adult; D005871:Castleman Disease / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D003937:Diagnosis, Differential; D004487:Edema / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D054969:Primary Dysautonomias / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D011877:Radionuclide Imaging; D016706:Still's Disease, Adult-Onset / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D013577:Syndrome
keywords: Adult-onset Still's disease; Dysautonomia; Dysautonomie; Maladie de Castleman multicentrique; Maladie de Still de l’adulte; Microangiopathie thrombotique; Multicentric Castleman's disease; Syndrome TAFRO; TAFRO syndrome; Thrombotic thrombocytopenic purpura