Liver involvement in Langerhans' cell histiocytosis: A study of nine cases

Nine cases of Langerhans' cell histiocytosis (LCH) of the liver are present ed. Five of the patients had liver involvement only. Other organ systems, n otably the lymph nodes and skin, were involved in the other four patients. Four of the patients had sclerosing biliary disease with infiltration of th e bile ducts by Langerhans' cells, whereas in two other patients, the bilia ry sclerosis was not associated with direct hepatic involvement by Langerha ns' cells. Histologically, the lesions were composed of focal aggregates of Langerhans' cells in a polymorphous background of mature eosinophils, lymp hocytes, neutrophils, and plasma cells. LCH encompasses a syndrome that has a broad range of clinical presentations and that might involve the liver s olely as tumor-like lesions or cystic lesions, or as part of systemic disea se. Even when Langerhans' cells are not demonstrable, sclerosing cholangiti s can be seen in LCH.