Langerhans cell histiocytosis in an adult patient associated with sclerosing cholangitis and cerebellar atrophy

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...

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Bibliographic Details
Published in:Gastroentérologie clinique et biologique Vol. 29; no. 3; p. 300
Main Authors: Desrame, Jérôme, Béchade, Dominique, Defuentes, Gilles, Goasdoue, Pascal, Raynaud, Jean-Jacques, Claude, Valère, Renard, Jean-Luc, Genereau, Thierry, Coutant, Gilles, Algayres, Jean-Pierre
Format: Journal Article
Language:French
Published: France 01-03-2005
Subjects:
Aged
Atrophy - etiology
Cerebellum - pathology
Cholangitis, Sclerosing - complications
Cholangitis, Sclerosing - etiology
Fatal Outcome
Histiocytosis, Langerhans-Cell - diagnosis
Humans
Liver Failure - etiology
Male
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Summary: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.
ISSN:0399-8320
DOI:10.1016/S0399-8320(05)80767-5