Autoimmune hemolytic anemia (AIHA) is a collective termfor several diseases characterized by autoantibody-initiated destruction\nof red blood cells (RBCs). Exact subclassification is essential. We provide a review of the respective types of AIHA with emphasis\non mechanisms of RBC destruction, focusing in particular on complement involvement. Complement activation plays a definitive\nbut limited role in warm-antibody AIHA (w-AIHA), whereas primary cold agglutinin disease (CAD), secondary cold agglutinin\nsyndrome (CAS), and paroxysmal cold hemoglobinuria (PCH) are entirely complement-dependent disorders. The details of\ncomplement involvement differ among these subtypes. The theoretical background for therapeutic complement inhibition in\nselected patients is very strong in CAD, CAS, and PCH but more limited in w-AIHA. The optimal target complement component\nfor inhibition is assumed to be important and highly dependent on the type of AIHA. Complement modulation is currently not\nan evidence-based therapy modality in any AIHA, but a number of experimental and preclinical studies are in progress and a few\nclinical observations have been reported. Clinical studies of new complement inhibitors are probably not far ahead.
Loading....