Giant cell hepatitis associated with autoimmune hemolytic anemia (GCH-AIHA) is a rare
disorder with unfavorable prognosis, affecting infants and young children. The mortality
rate is high, complications of acute liver failure, sepsis, or liver transplantation
can be responsible for fatal outcomes. An 18-month-old child who was diagnosed previously
with autoimmune hemolytic anemia, developed acute hepatitis and acute liver failure
concomitant to the relapse of the disease. GCH-AIHA is characterized by Coombs positive
hemolytic anemia and progressive liver injury, histologically defined by widespread
giant cell transformation. Liver biopsy was performed to establish the diagnosis,
histological examination confirmed the presence of multinuclear, giant cell hepatocytes.
Corticosteroid and azathioprine treatment were started. As a result of subsequent
rituximab treatment and intravenous immunoglobulin therapy, acute liver failure and
anemia gradually resolved. The exact background of the association of the two entities
is still unknown, an autoimmune mechanism is suspected. Conventional immunosuppressive
treatment with corticosteroid and azathioprine seems to be ineffective in most cases,
therefore second- and third-line therapies are required. Since the introduction of
the anti-CD20 rituximab therapy, the prognosis of GCH-AIHA has improved significantly.
Orv Hetil. 2023; 164(36): 1432-1436.
