Liver transplantation in an adult with sclerosing cholangitis due to Langerhans cell histiocytosis

JOURNAL OF HEPATOLOGY

卷:44

Article

Griffiths, W ; Davies, S ; Gibbs, P ; Thillainayagam, A ; Alexander, G

关键词: Langerhans cell histiocytosis;    sclerosing cholangitis;    liver transplantation;   

DOI  :  10.1016/j.jhep.2005.12.024

来源: Elsevier

【 摘 要 】

Sclerosing cholangitis due to Langerhans cell histiocytosis (LCH) is a rare cause of end-stage liver disease, seen mainly in children. Only a few adult cases have been reported worldwide. Liver transplantation may be a viable treatment option for what is otherwise an irreversible condition. We describe a 65-year-old female with LCH who developed severe sclerosing cholangitis with jaundice, intractable pruritus and peritoneal disease. She underwent orthotopic liver transplantation with complete amelioration of symptoms and remained well 14 months following her operation. Explant histology confirmed LCH involvement with an associated extensive sclerosing cholangitis. Symptomatic LCH cholangiopathy is an emerging indication for liver transplantation in adults. (c) 2006 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

【 授权许可】

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