Portal hypertension secondary to Langerhans cell histiocytosis

We report two children, aged 2 and 2 1/2 years, with multisystem Langerhans cell histiocytosis (LCH). Both were administered chemotherapy, with apparently good response. However, hepatic fibrosis and portal hypertension were detected 5 and 1 1/2 years after therapy, respectively. The first child died after a bout of hematemesis. Hepatic fibrosis can proceed despite apparently successful chemotherapy in LCH.