Skip Navigation LinksHome > May 2014 - Volume 58 - Issue 5 > Giant Cell Hepatitis With Autoimmune Hemolytic Anemia in Chi...
Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0000000000000270
Original Article: Hepatologyand Nutrition

Giant Cell Hepatitis With Autoimmune Hemolytic Anemia in Children: Proposal for Therapeutic Approach

Bakula, Agnieszka*; Socha, Piotr*; Klaudel-Dreszler, Maja*; Karolczyk, Grazyna; Wozniak, Malgorzata*; Rutynowska-Pronicka, Olga; Matysiak, Michal§

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Abstract

Background and Objective: Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AIHA) is a rare, progressive disorder in infants and young children. The disease is aggressive and may lead to liver or multiorgan failure with fatal prognosis. Therapy with anti-CD20 monoclonal antibody, rituximab (Rtx), proved effective. The primary objective of the study was to evaluate therapy for severe GCH with AIHA.

Methods: We report on 5 cases of severe GCH with AIHA treated in our department between 2006 and 2011.

Results: The median age of the children was 7 months (2–12 months), follow-up lasted 44 months (12–78 months), median (min-max), and the main observed symptoms were jaundice and hepatosplenomegaly. All of the children had positive direct Coombs test and biopsy-proven giant cell transformation of hepatocytes. Liver failure was observed in 3 children. First-line therapy (prednisone, azathioprine) proved ineffective in all but 1 of the patients, who initially responded to the treatment but relapsed after 4 months. The child subsequently developed hemophagocytic lymphohistiocytosis and died 2 months after hemopoietic stem cell transplantation. Four remaining patients finally achieved complete remission after 4 to 6 doses of Rtx.

Conclusions: We propose Rtx as the treatment of choice for severe GCH with AIHA in the early stages of the disease, provided steroids and azathioprine are ineffective.

© 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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