Successful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab
10.5223/pghn.2020.23.2.180
- Author:
Young Ho KIM
1
;
Ju Whi KIM
;
Eun Joo LEE
;
Gyeong Hoon KANG
;
Hyoung Jin KANG
;
Jin Soo MOON
;
Jae Sung KO
Author Information
1. Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. kojs@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Hepatitis;
Autoimmune hemolytic anemia;
Rituximab
- MeSH:
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
- From:Pediatric Gastroenterology, Hepatology & Nutrition
2020;23(2):180-187
- CountryRepublic of Korea
- Language:English
-
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.
