Autoimmune Hemolytic Anemia (AIHA) is a heterogeneous group of rare, acquired immune disorders characterized by autoantibody-mediated destruction of red blood cells (RBC) resulting in anemia. The incidence in adults has been estimated at 1-3 cases per 100,000/year.
The pathogenesis of AIHA involves the production of IgG and IgM autoantibodies that bind to RBC surface antigens, initiating red blood cell destruction via the complement system and reticuloendothelial system. The complement system plays an important role in mediating cell lysis. Patients with AIHA usually present with symptoms of anemia. In cases of severe hemolysis, there may be fever, pallor, jaundice, hepatosplenomegaly, hemoglobinuria and signs of heart failure and/or renal failure, and death has been reported in up to 11% of patients (Lechner and Jager, 2010).
Glucocorticoids continue to be considered as first-line treatment, but improvement is slow and relapses are common. Complications from corticosteroids are of particular concern in patients with underlying diabetes. For those who fail to respond or who relapse, splenectomy may be considered.
RLS-0088 and RLS-00XX have the potential to stabilize AIHA patients more rapidly than glucocorticoids without the associated negative side-effects.
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Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances foreign to the body.
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