Introduction:

giant cell hepatitis with autoimmune hemolytic anemia (GCH-AIHA) is a rare and serious condition that usually occurs during the first year of life. There is no specific diagnostic test; it is histologically characterized by a transformation of hepatocytes into giant cells.

Case presentation:

male patient who at 45 days-old starts with jaundice, pallor, and hepatomegaly. Leukocytosis, normocytic anemia with positive direct Coombs, and elevation of bilirubin, lactate dehydrogenase and transaminases were found. Serology for syphilis, toxoplasma, rubella, cytomegalovirus, and herpes simplex were negative. Diagnosis was established by histopathological findings in liver biopsy, plus hemolytic anemia. The patient was treated with steroids and azathioprine, and has maintained a satisfactory evolution until the age of eight years, without relapses, and improvement of the histopathological pattern.

Conclusions:

GCH-AIHA is a serious disease, which should be suspected in infants with hemolytic anemia, plus evidence of acute hepatitis, after ruling out the main causes of hepatitis.

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