Coombs-positive autoimmune hemolytic anemia and postinfantile giant cell hepatitis in children.

We report a 23-month-old girl and a 9-month-old boy who presented with autoimmune hemolytic anemia followed by recurrent episodes of severe hepatitis. The first episode of hepatitis occurred 1 week and 15 months after presentation, respectively. Histologically, the livers showed loss of lobular architecture with diffuse giant cell transformation of hepatocytes and portal and pericellular fibrosis. The first patient died at 4 1/2 months after her initial presentation with a well-established micronodular cirrhosis. The second patient responded to steroid therapy and the hepatitis recurred when steroids were tapered. Postinfantile giant cell hepatitis may occur in association with Coombs-positive hemolytic anemia, it is thought to have an autoimmune mechanism, and early and sustained immunosuppression may control the progressive hepatocellular damage and prevent cirrhosis.