title: Giant cell hepatitis with autoimmune hemolytic anemia in early childhood: long-term outcome in 16 children.
abstract: OBJECTIVE
  To assess the outcome of giant cell hepatitis combined with autoimmune hemolytic anemia in early childhood.
  
  
  METHODS
  We report on 16 children with this disease evaluated over a 28-year period.
  
  
  RESULTS
  Children (nine boys; median age, 6 months) presented with jaundice, hepatomegaly, elevated aminotransferases, a positive Coombs test, and diffuse giant-cell transformation of hepatocytes on histology. Treatment with prednisone and azathioprine, plus, in three children, cyclosporine, resulted in complete remission in eight, partial remission in six, and failure in two. Relapses of hepatitis and/or anemia occurred in 11 and 10 children, respectively, requiring prolonged high levels of immunosuppression, and splenectomy or Rituximab, respectively. Treatment was stopped after a mean duration of 6 years, with no relapse, in seven children, with a median follow-up of 14 years. One child is alive 9 years after liver transplantation. Four children died of sepsis or multiple organ failure.
  
  
  CONCLUSIONS
  Giant cell hepatitis combined with autoimmune hemolytic anemia requires rigorous treatment. Immunosuppressive therapy results in remission in most cases. A complete cure can be expected after several years of intensive treatment. Liver transplantation may be associated with prolonged survival.
authors.affiliation: Department of Pediatrics and Division of Pediatric Gastroenterology and Hepatology, University Hospital Santa Chiara, Pisa, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Maggiore
authors.forename: Marco
authors.initials: M
authors.lastname: Sciveres
authors.forename: Monique
authors.initials: M
authors.lastname: Fabre
authors.forename: Laura
authors.initials: L
authors.lastname: Gori
authors.forename: Lucia
authors.initials: L
authors.lastname: Pacifico
authors.forename: Massimo
authors.initials: M
authors.lastname: Resti
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Choulot
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Jacquemin
authors.forename: Olivier
authors.initials: O
authors.lastname: Bernard
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000401:mortality / Q000628:therapy*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D001663:Bilirubin / Q000097:blood; D003298:Coombs Test; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D015726:Giant Cells / Q000473:pathology*; D005938:Glucocorticoids / Q000627:therapeutic use; D006505:Hepatitis / Q000150:complications* / Q000401:mortality / Q000628:therapy*; D022781:Hepatocytes / Q000473:pathology; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007565:Jaundice / Q000209:etiology; D008099:Liver / Q000473:pathology*; D016031:Liver Transplantation; D008297:Male; D009102:Multiple Organ Failure / Q000401:mortality; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab; D018805:Sepsis / Q000401:mortality; D013156:Splenectomy; D013163:Splenomegaly / Q000209:etiology; D000637:Transaminases / Q000097:blood; D016896:Treatment Outcome; D005719:gamma-Globulins / Q000032:analysis
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D005938:Glucocorticoids; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D005719:gamma-Globulins; D000069283:Rituximab; D016572:Cyclosporine; D000637:Transaminases; D001379:Azathioprine; D001663:Bilirubin; D011241:Prednisone