A Case of Secondary Sclerosing Cholangitis Leading to Liver Cirrhosis Due to Langerhans Cell Histiocytosis : Successful Treatment with Living Related Liver Transplantation

Abstract

We reported a case of secondary sclerosing cholangitis due to Langerhans' cell histiocytosis (LCH), who received living related liver transplantation for liver cirrhosis. The patient was found to have hepatomegaly and liver dysfunction on follow-up examination of uveitis. The diagnosis of sclerosing cholangitis was made by CT scan, MRI, cholangiography, and liver biopsy. Since he had repetitive cholangitis-like symptoms including fever and abdominal pain, he underwent excision of the dilated extrahepatic bile duct and choledocho-jejunostomy. Pathological examination revealed severe liver cirrhosis and marked involvement of Langerhans' cells in submucosal layer of the bile ducts. Because of repetitive cholangitis and severe liver cirrhosis with esophageal varices, he received living related liver transplantation from his mother. He experienced no recurrence of LCH for nine months after liver transplantation. Liver transplantation should be positively considered for treatment of liver cirrhosis associated with LCH.