Abstract
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis. LCH diseases may occur in a single organ or multisystem organ. The patients with multisystem involvement usually have a poor prognosis. Liver involvement in multisystem LCH results in severe complications, such as obvious sclerosing cholangitis (SC) with jaundice. METHODS: We reported a 31-year-old man developed severe SC due to multisystem LCH and was successfully treated by liver transplantation (LT). In addition, we firstly used tacrolimus and mycofenolate mofetil as immunosuppressants to treat LCH after LT. RESULTS: We performed the immunosuppressants to deal with the LCH after LT, now the patient is currently well with normal liver function and no evidence of recurrence of LCH for 4 and a half years follow-up. CONCLUSION: LT should be recommended as an effective treatment for these adults with severe SC due to multisystem LCH. Finally, using tacrolimus and mycofenolate mofetil as immunosuppressants to treat LCH might be favorable to prevent LCH recurrence.