INVESTIGATING THE MECHANISMS AND THERAPEUTIC CHALLENGES OF HEMOLYTIC ANEMIA LINKED TO GIANT CELL HEPATITIS

Abstract

The pathogenesis of hemolytic anemia associated with giant cell hepatitis (GCH) in the conte Giant cell hepatitis (GCH) associated with autoimmune hemolytic anemia (AHA) is a rare but severe pediatric condition with high mortality rates. This report presents the case of a nine-month-old infant with jaundice, fever, and watery diarrhea, who rapidly developed severe hepatic failure. Laboratory findings included normocytic anemia, thrombocytopenia, elevated alpha-fetoprotein (AFP), and a positive direct Coombs' test (IgG and C3d). Despite ruling out infectious and metabolic causes, GCH with AHA was diagnosed following a liver biopsy revealing giant necrotic hepatocytes.  The patient received intravenous immunoglobulin (IVIG), prednisone, and azathioprine, which initially stabilized clinical parameters. However, a severe relapse occurred within four months, presenting with multi-drug-resistant Escherichia coli sepsis, ascites, and worsening liver failure. The infant ultimately succumbed to septic shock despite intensive supportive care. This case highlights the diagnostic challenges and aggressive nature of GCH associated with AHA. The rapid relapse following partial remission emphasizes the need for prolonged immunosuppressive therapy and early intervention with liver biopsy in cases of unexplained hepatitis. Additionally, it underscores the limitations of current treatments, including corticosteroids, IVIG, and splenectomy, in preventing relapse. GCH with AHA remains a life-threatening condition with poor outcomes, necessitating continued research to identify effective therapies and predictors of relapse. This case further reinforces the importance of close monitoring and comprehensive long-term management strategies to improve patient survival.xt of autoimmune hemolysis remains elusive. A 9-month-old infant presented with fever, diarrhea, and jaundice four days prior to hospitalization. Physical examination revealed pallor, jaundice, and hepatosplenomegaly. Laboratory findings indicated elevated levels of hemolytic anemia, thrombocytopenia, immunoglobulin G (IgG), and anti-C3d antibodies. Conjugated bilirubin was measured at 84 mmol/L, with a total bilirubin of 101mmol/L. The absence of antinuclear antibodies, anti-smooth muscle antibodies, anti-liver kidney microsomes 1 antibodies, and anti-endomisium antibodies, as well as negative results for Epstein Barr virus, cytomegalovirus, herpes simplex, and viral hepatitis B and C, led to the diagnosis of GCH. GCH diagnosis was supported by acute liver failure, Evan's syndrome, and positive Coomb's tests (IgG and C3). Confirmation of GCH was obtained via needle liver biopsy. Despite treatment with steroids, immune-modulatory therapy, and azathioprine, the patient succumbed to the condition.