Pediatr Gastroenterol Hepatol Nutr.  2020 Mar;23(2):180-187. 10.5223/pghn.2020.23.2.180.

Successful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab

Affiliations
  • 1Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. kojs@snu.ac.kr
  • 2Department of Pathology, Seoul National University Hospital, Seoul, Korea.
  • 3Department of Pediatrics, Seoul National University College of Medicine, Seoul National University Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.

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.

Keyword

Hepatitis; Autoimmune hemolytic anemia; Rituximab

MeSH Terms

Anemia, Hemolytic
Anemia, Hemolytic, Autoimmune*
Biopsy
Giant Cells*
Hepatitis*
Hepatocytes
Humans
Infant*
Inflammation
Jaundice
Liver
Liver Diseases
Male
Pallor
Physical Examination
Rare Diseases
Recurrence
Rituximab*
Splenomegaly
Rituximab
Full Text Links
  • PGHN
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
    DB Error: unknown error