A 51-year-old woman presented with superficial thrombophlebitis and severe hemolytic anemia. The initial direct antiglobulin test using a broad-spectrum antiserum for screening was negative. Later testing with monospecific antisera revealed the rare finding of an autoimmune hemolytic anemia (AIHA) associated with IgA autoantibody. In IgA-AIHA the direct antiglobulin test with a broad-spectrum antiserum may be falsely negative, because a standardized anti-IgA level is not compulsory. This case illustrates the diagnostic significance of monospecific antisera in IgA-AIHA. Our patient responded well to high-dose steroids, but due to repeated exacerbations this treatment could not be tapered. Splenectomy was performed 19 months later. Since then the AIHA remained in remission, even after steroids were stopped, and although the direct antiglobulin test continued to be positive for IgA and became positive again for C3d. Available data about pathogenesis and therapy of IgA-AIHA are reviewed.

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