Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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Successful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab

  • Kim, Young Ho (Department of Pediatrics, Seoul National University Hospital) ;
  • Kim, Ju Whi (Department of Pediatrics, Seoul National University Hospital) ;
  • Lee, Eun Joo (Department of Pediatrics, Seoul National University Hospital) ;
  • Kang, Gyeong Hoon (Department of Pathology, Seoul National University Hospital) ;
  • Kang, Hyoung Jin (Department of Pediatrics, Seoul National University College of Medicine, Seoul National University Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Moon, Jin Soo (Department of Pediatrics, Seoul National University Hospital) ;
  • Ko, Jae Sung (Department of Pediatrics, Seoul National University Hospital)
  • Received : 2019.09.30
  • Accepted : 2020.01.28
  • Published : 2020.03.15
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Abstract

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

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References

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  1. Infantile giant cell hepatitis with autoimmune hemolytic anemia vol.13, pp.4, 2020, https://doi.org/10.4254/wjh.v13.i4.411