Liver transplantation in an adult with sclerosing cholangitis due to Langerhans cell histiocytosis.
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.
Department of Hepatology, Addenbrooke's Hospital, Box 210, Cambridge CB2 2QQ, UK. bill.griffiths@addenbrookes.nhs.uk
William
W
Griffiths
Susan
S
Davies
Paul
P
Gibbs
Andrew
A
Thillainayagam
Graeme
G
Alexander
D000368:Aged; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000209:etiology* / Q000473:pathology / Q000601:surgery*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation