Background and aim: Autoimmune hemolytic anemias (AIHA) constitute a rare and heterogeneous group of diseases whose therapy differs according to the type of antibody involved in the genesis of the disease and the existence or not of an identified cause. With the aim of providing a practical guide for the therapy of AIHA, we summarize the emergency therapy and general measures habitually used in all forms of AIHA, as well as the specific treatment of the most frequent primary forms of AIHA: primary warm AIHA and AIHA from cold agglutinin disease (AIHA from CAD). We discuss the dependence of the treatment of the secondary forms on their underlying causes and the changes in the treatment of the primary forms in recent years.
Methods: We examined the options available for the treatment of primary warm AIHA and AIHA from CAD.
Results: We found many differences and only one similarity in their treatment.
Discussion: The differences, particularly due to the non-responsiveness of AIHA from CAD to many treatments useful for primary warm AIHA, such as steroids, splenectomy and immunosuppressive agents, must be considered in the face of each, single case of AIHA. Preliminary identification of the type of antibody involved in the genesis of the disease and careful exclusion of a secondary form are particularly important. Rituximab plays a central role in the treatment of primary warm AIHA and AIHA from CAD.
Keywords: Autoimmune hemolytic anemia; Cold agglutinin disease; Therapy; Warm autoantibodies.
© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.