Abstract
We report the death for liver cirrhosis of a boy who contracted Langerhans cell histiocytosis at the age of three. On admission, laboratory data revealed cholestasis. Despite chemotherapy, abnormal liver function tests persisted. Two months after admission, needle liver biopsy showed relatively massive fibrosis and proliferation of bile ductules with lymphocyte infiltration in the portal areas. Five years after onset of disease, recurrent bleeding from esophagogastric varices occurred, and endoscopic sclerotherapy was done a total of 10 times. Although living related liver transplantation was considered, it could not be done because of several factors. Thirteen years after onset of disease, ascites and coagulation abnormality developed. Two years later, he suddenly died with acute respiratory distress. Autopsy revealed pulmonary bleeding and liver cirrhosis.
