[Langerhans cell histiocytosis in an adult patient associated with sclerosing cholangitis and cerebellar atrophy].
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.
Desrame
Jérôme
J
Clinique médicale, Hôpital d'Instruction des Armées Val de Grâce, 74 Bd de Port Royal, 75005 Paris. capgast@club-internet.fr
Béchade
Dominique
D
Defuentes
Gilles
G
Goasdoue
Pascal
P
Raynaud
Jean-Jacques
JJ
Claude
Valère
V
Renard
Jean-Luc
JL
Genereau
Thierry
T
Coutant
Gilles
G
Algayres
Jean-Pierre
JP
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