Recurrence of langerhans cell histiocytosis in the graft after pediatric liver transplantation

Two girls were diagnosed with Langerhans cell histiocytosis (LCH) at the ag e of 16 and 7 months and developed end stage chronic liver disease related to LCH-induced sclerosing cholangitis at 28 and 8 months, respectively. The y received liver transplants at 34 and 14 months of age. Five months post-o rthotopic liver transplantation (OLT) one of the patients developed posttra nsplant lymphoproliferative disease, successfully treated with a combinatio n of surgery and reduction of immunosuppression. Fourteen months post-OLT s he developed diabetes insipidus, bilateral ear discharge, and new osteolyti c lesions. After transplantation both girls had mild skin reactivations of LCH, requiring minimal steroid increments. At 60 and 5 months post-OLT intr ahepatic LCH recurrence was diagnosed on the basis of abnormal biliary enzy mes and presence of Langerhans cells in the grafts. Initial cholangiography in both patients was unremarkable. LCH activity was controlled by maintena nce chemotherapy with vinblastine, etoposide, and prednisolone. Ten months after reappearance of LCH in the liver graft a follow-up cholangiography in one of the girls demonstrated a low grade cholangiopathy. Residual elevati on of liver enzymes probably represents an ongoing pathogenic process.