Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood

Four children, aged 61/2 months to 2 years, presented with liver disease and autoimmune hemolyticanemia. Clinical signs included fever, jaundice, firm or hard hepatomegaly, and splenomegaly. Direct Coombs test results were of the mixed (IgG+C) type. Liver function tests showed high direct bilirubin, transaminase, and serum gamma globulin values, and a prolonged prothrombin time. The liver histology was characterized by marked lobular fibrosis and giant cell transformation. The course of the disease was severe, resulting in the death of three patients from liver failure. However, the liver disease seemed responsive to corticosteroid treatment, which in one patient was clearly beneficial.