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

Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder characterized by proliferation of CD1a+ dendritic cells. An association between LCH and sclerosing cholangitis (SC) has rarely been reported in adults with only four cases of liver transplantation for LCH associated SC in the literature (1). We have recently managed such a patient with SC secondary to LCH who eventually required liver transplantation, and feel the case adds to the evidence that liver transplantation can be efficacious in treating some rare diseases, such as LCH, and that posttransplant immunosuppression can prevent recurrence. Furthermore, it highlights the ethical implications of using valuable organs when evidence for transplant is scarce.