title: [Langerhans cell histiocytosis in an adult patient associated with sclerosing cholangitis and cerebellar atrophy].
abstract: Langerhans' cell histiocytosis is a disorder in children or young adults, characterized by clonal proliferation of histiocytic cells, staining for CD1a, with uni or multifocal organ involvement. It's a rare condition in adults. We report a case of Langerhans' cell histiocytosis in an adult with sclerosing cholangitis which rapidly progressed to fatal liver failure and progressive cerebellar atrophy. Langerhans cell histiocytosis is a rare cause of sclerosing cholangititis in adults.
authors.affiliation: Clinique médicale, Hôpital d'Instruction des Armées Val de Grâce, 74 Bd de Port Royal, 75005 Paris. capgast@club-internet.fr
authors.forename: Jérôme
authors.initials: J
authors.lastname: Desrame
authors.forename: Dominique
authors.initials: D
authors.lastname: Béchade
authors.forename: Gilles
authors.initials: G
authors.lastname: Defuentes
authors.forename: Pascal
authors.initials: P
authors.lastname: Goasdoue
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Raynaud
authors.forename: Valère
authors.initials: V
authors.lastname: Claude
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Renard
authors.forename: Thierry
authors.initials: T
authors.lastname: Genereau
authors.forename: Gilles
authors.initials: G
authors.lastname: Coutant
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Algayres
mesh_terms: D000368:Aged; D001284:Atrophy / Q000209:etiology; D002531:Cerebellum / Q000473:pathology*; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000209:etiology*; D017809:Fatal Outcome; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis*; D006801:Humans; D017093:Liver Failure / Q000209:etiology; D008297:Male