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

Abstract

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.

Autoimmune hemolytic anemia in an infant with giant cell hepatitis

“…The liver biopsy in both of these patients showed characteristics giant cell transformation of the hepatocytes. It is thus possible that the syndrome described by Bernard et al and other researchers [1][2][3][4] is part of spectrum of manifestation of autoimmune hepatitis and not a distinct syndrome by itself.…”

“…Autoimmune hemolytic anemia associated with GCH is a rare entity [1][2][3][4]. Positive direct Coombs test is the only sign of autoimmunity in these patients.…”

“…The liver histopathology shows giant cell transformation, lobular fibrosis, disruption of lobular architecture, parenchymal collapse, and mild portal inflammatory cell infiltration. The histopathology does not meet the criteria for diagnosis of autoimmune hepatitis [1][2][3]. The serological test for anti-mitochondrial, anti-smooth muscle, anti-liver kidney microsomal (LKM-1), and anti-nuclear antibodies are negative, and this disorder is considered to be a distinct syndrome.…”

“…Patients who do not respond to steroids may require immunosuppression with cyclophosphamide or azathioprine. However, relapses are frequent on withdrawal of immunosuppressive therapy [1][2][3].…”

“…Autoimmune hemolytic anemia (AIHA) in association with giant cell hepatitis (GCH) is a rare disorder, and only a few cases have been reported in infants [1][2][3][4]. The liver disease in these patients, usually has an aggressive course leading to hepatic failure, cirrhosis, and ultimately death.…”