INFANTILE GIANT CELL HEPATITIS WITH AUTOIMMUNE HEMOLYTIC ANEMIA
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Date
2021-04-27
Authors
Poddighe, Dimitri
Madiyeva, Aidana
Talipova, Diana
Umirbekova, Balzhan
Journal Title
Journal ISSN
Volume Title
Publisher
World Journal of Hepatology
Abstract
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.
Description
Keywords
Type of access: Open Access, Giant cell hepatitis, Autoimmune hemolytic anemia, Rituximab, Infantile hepatitis, Jaundice
Citation
Poddighe, D., Madiyeva, A., Talipova, D., & Umirbekova, B. (2021). Infantile giant cell hepatitis with autoimmune hemolytic anemia. World Journal of Hepatology, 13(4), 411–420. https://doi.org/10.4254/wjh.v13.i4.411