Liver transplantation in patients with Langerhans' cell histiocytosis.

Abstract

Langerhans’ cell histiocytosis (LCH)* is an uncommon disease, characterized by an abnormal proliferation of histiocytic cells of the dendritic family. Some patients with systemic LCH have primary liver involvement that may resemble sclerosing cholangitis. Others may develop end-stage liver disease as a result of the chemotherapeutic agents used to control the primary disorder. We have performed liver transplantation in two children and one adult with this disease, the largest single-center experience in the world. A follow-up of these cases may be of special interest for two reasons. First, perturbations in the immune system are common in this disease. Second, light can be shed on whether immunosuppression can influence a disease with a possible autoimmune etiology.