ABSTRACT
Autoimmune hemolytic anemia (AIHA) is a relatively uncommon autoimmune disease that is unique for two reasons: First, the diagnostic autoantibody for the disorder is the direct mediator of red cell destruction. Second, because both the autoantibody and its target (red cells) can easily be obtained, the antibody specificity and target antigen have been well characterized. While progress has been made in characterizing the target autoantigens and understanding the mechanism of autoantibody-mediated red cell destruction, much less is known about the underlying immune dysfunction that leads to the abnormal generation of the red cell autoantibodies.
