title: Case report and literature review: Glomerular and neurologic thrombotic microangiopathy as a primary manifestation of multicentric castleman disease.
abstract: BACKGROUND
  We report the case of a multicentric Castleman disease (MCD) with initial renal involvement. Although the renal involvement in this case was typical of MCD, it constitutes a rare presentation of the disease, and in our case the renal manifestations led to the haematological diagnosis.
  
  
  RESULTS
  The patient was admitted for fever, diarrhea, anasarca, lymphadenopathies and acute renal failure. Despite intravenous rehydration using saline and albumin, renal function worsened and the patient required dialysis. While diagnostic investigations were performed, right hemiplegia occurred. There was no anemia or thrombocytopenia.
  
  
  METHODS
  Kidney biopsy was consistent with glomerular thrombotic microangiopathy (TMA). Lymph node histology was consistent with hyalin-vascular variant of Castleman disease.
  
  
  RESULTS
  Given the renal and neurological manifestations of this MCD-associated TMA, the patient was treated with plasma exchange during one month, and six courses of rituximab, cyclophosphamide and dexamethasone. The evolution was favorable.
  
  
  CONCLUSIONS
  Although rare, this diagnosis is worth knowing, as specific treatment has to be started as soon as possible and proved to be efficient in our case as well as in other reports in the literature.
authors.affiliation: Department of Nephrology, Necker-Enfants Malades Hospital, Paris, France College de France, Laboratory of Central Neuropeptides in the Regulation of Body Fluid Homeostasis and Cardiovascular Functions, Center for Interdisciplinary Research in Biology (CIRB), Paris, France Department of Pathology, Necker-Enfants Malades Hospital, Paris, France Department of Hematology, Necker-Enfants Malades Hospital, Paris, France.
authors.forename: Adrien
authors.initials: A
authors.lastname: Flahault
authors.forename: Marguerite
authors.initials: M
authors.lastname: Vignon
authors.forename: Marion
authors.initials: M
authors.lastname: Rabant
authors.forename: Aurélie
authors.initials: A
authors.lastname: Hummel
authors.forename: Laure-Hélène
authors.initials: LH
authors.lastname: Noël
authors.forename: Danielle
authors.initials: D
authors.lastname: Canioni
authors.forename: Bertrand
authors.initials: B
authors.lastname: Knebelmann
authors.forename: Felipe
authors.initials: F
authors.lastname: Suarez
authors.forename: Khalil
authors.initials: K
authors.lastname: El Karoui
mesh_terms: D001706:Biopsy; D005871:Castleman Disease / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D006801:Humans; D007678:Kidney Glomerulus / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000209:etiology*