Abstract

The diagnosis of autoimmune haemolytic anaemia (AIHA) is a challenge for both the immune-haematology laboratory and the clinician as the laboratory investigation can be troublesome and often requires extensive time-consuming serological testing, especially when a blood transfusion is needed. Frequently, there is a need to start therapy rapidly. Autoantibodies directed to epitopes on red blood cell (RBC) consisting in sugar and/or protein structures are crucial in the pathogenesis of AIHA. The isotype is important for the clinical significance of an autoantibody. Frequently, patients are icteric and suffer from clinical signs of anaemia, such as pallor, fatigue, shortness of breath and palpitations, on examination hepatosplenomegaly can be present. If there is no vital indication for a transfusion it is prudent to wait for the results of the immune-haematological tests and the ensuing transfusion advice based on this. Focus of treatment should be stoppage of hemolysis or at least be attenuated via an inhibition of autoantibody production and/or inhibition of premature RBC destruction. Successful treatment of secondary AIHA is only possible when the underlying disease is treated.

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