Giant cell hepatitis and autoimmune hemolytic anemia: efficacy of splenectomy on hemolysis

The previously reported cases of giant cell hepatitis with autoimmune hemolytic anemia were improved by prednisone plus azathioprine. A 14-month-old boy suffered from giant cell hepatitis with auto-immune hemolytic anemia and positive direct Coombs test. Prednisone and azathioprine administration improved the liver disease but failed to control hemolysis so that repeated blood transfusions were necessary. Persistance of severe degree of hemolysis required splenectomy that was promptly and definitively effective. Azathioprine and prednisone were pursued for a total duration of five years. Twelve years after the onset of the disease, the child is well without any treatment. This is the first reported case of such an association in which poorly controlled auto-immune hemolytic anemia benefited from splenectomy.