J Pediatr 1981 Nov;99(5):704-11

Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood

Bernard O, Hadchouel M, Scotto J, Odievre M, Alagille D

Abstract
Four children, aged 6 1/2 months to 2 years, presented with liver disease and
autoimmune hemolytic anemia. 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