Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy

Eur J Pediatr. 1991 Mar;150(5):314-7. doi: 10.1007/BF01955929.


An 8-month-old boy and a 7-month-old girl presented with an acute, Coombs-positive auto-immune haemolytic anaemia and severe hepatitis. The clinical manifestations were pallor, jaundice and hepatomegaly. The liver histology revealed diffuse giant cell transformation and extensive necrosis with central-portal bridging. Combined immunosuppressive regimen with steroids and azathioprine led to prolonged clinical and biological remission with a respective 2 years and 7 months follow up. The girl, however, after 7 months developed a progressive encephalopathy of unknown aetiology, while liver and haematological disease were still under control. She died subsequently from severe recurrent seizures. We conclude that acute Coombs-positive giant cell hepatitis of infancy can be improved by sustained immunosuppressive therapy.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Adrenal Cortex Hormones / therapeutic use*
  • Anemia, Hemolytic, Autoimmune / complications
  • Anemia, Hemolytic, Autoimmune / drug therapy*
  • Azathioprine / therapeutic use*
  • Coombs Test*
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Hepatitis / complications
  • Hepatitis / drug therapy*
  • Hepatitis / pathology
  • Humans
  • Infant
  • Male
  • Necrosis


  • Adrenal Cortex Hormones
  • Azathioprine