Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Apr 27, 2021; 13(4): 411-420
Published online Apr 27, 2021. doi: 10.4254/wjh.v13.i4.411
Infantile giant cell hepatitis with autoimmune hemolytic anemia
Dimitri Poddighe, Aidana Madiyeva, Diana Talipova, Balzhan Umirbekova
Dimitri Poddighe, Aidana Madiyeva, Diana Talipova, Department of Medicine, Nazarbayev University School of Medicine, Nur-Sultan 010000, Kazakhstan
Dimitri Poddighe, Balzhan Umirbekova, Department of Pediatrics, National Research Center for Maternal and Child Health, Nur-Sultan 010000, Kazakhstan
Author contributions: Poddighe D conceived and wrote the manuscript; Madiyeva A and Talipova D contributed to drafting the manuscript and reviewing the relevant literature; Poddighe D and Umirbekova B provided intellectual contribution.
Conflict-of-interest statement: The authors have no conflict of interest to declare.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Dimitri Poddighe, MD, MSc, PhD-eq, Associate Professor, Department of Medicine, Nazarbayev University School of Medicine, Kerei-Zhanibek Str. 5/1, Nur-Sultan 010000, Kazakhstan.
Received: January 9, 2021
Peer-review started: January 9, 2021
First decision: January 25, 2021
Revised: January 26, 2021
Accepted: March 8, 2021
Article in press: March 8, 2021
Published online: April 27, 2021

Giant cell hepatitis (GCH) is characterized by large and multinucleated (syncytial) hepatocytes in the context of liver inflammation. Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity. The etiology is unknown; concomitant viral infections (as potential trigger factors) have been identified in a few patients. The pathogenesis reportedly relies upon immune-mediated/ autoimmune mechanisms. This condition should be considered in any infant developing Coombs-positive anemia; indeed, anemia usually precedes the development of hepatitis. The clinical course is usually aggressive without the appropriate immunosuppressive therapy, which may include steroids, conventional immunosuppressors (e.g., azathioprine and cyclophosphamide as first-line treatments), intravenous immunoglobulin, and biologics (rituximab). Improvements in medical management (including the availability of rituximab) have significantly reduced the mortality of this condition in the last decade.

Keywords: Giant cell hepatitis, Autoimmune hemolytic anemia, Rituximab, Infantile hepatitis, Jaundice, Hyperbilirubinemia

Core Tip: This review discusses the main characteristics of giant cell hepatitis associated with autoimmune hemolytic anemia including etiology, pathogenesis, pathophysiology, clinical aspects, prognosis, and therapy. All of the available case reports and case series have been considered to provide an overall picture of this disease and its general clinical management.