Infantile giant cell hepatitis with autoimmune hemolytic anemia

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.

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.