Abstract
Autoimmune hemolytic anemia (AIHA) is a collective term for several diseases characterized by autoantibody-initiated destruction of red blood cells (RBCs). Exact subclassification is essential. We provide a review of the respective types of AIHA with emphasis on mechanisms of RBC destruction, focusing in particular on complement involvement. Complement activation plays a definitive but limited role in warm-antibody AIHA (w-AIHA), whereas primary cold agglutinin disease (CAD), secondary cold agglutinin syndrome (CAS), and paroxysmal cold hemoglobinuria (PCH) are entirely complement-dependent disorders. The details of complement involvement differ among these subtypes. The theoretical background for therapeutic complement inhibition in selected patients is very strong in CAD, CAS, and PCH but more limited in w-AIHA. The optimal target complement component for inhibition is assumed to be important and highly dependent on the type of AIHA. Complement modulation is currently not an evidence-based therapy modality in any AIHA, but a number of experimental and preclinical studies are in progress and a few clinical observations have been reported. Clinical studies of new complement inhibitors are probably not far ahead.
Highlights
Autoimmune hemolytic anemia (AIHA) is a heterogeneous group of disorders characterized by autoantibody-mediated destruction of red blood cells (RBCs) [1,2,3]
We provide a review of the respective types of AIHA with emphasis on mechanisms of RBC destruction, focusing in particular on complement involvement
Complement modulation is currently not an evidence-based therapy modality in any AIHA, but a number of experimental and preclinical studies are in progress and a few clinical observations have been reported
Summary
Autoimmune hemolytic anemia (AIHA) is a heterogeneous group of disorders characterized by autoantibody-mediated destruction of red blood cells (RBCs) [1,2,3]. During the last five decades we have learned a great deal about the essential role of complement in subgroups of AIHA [6,7,8]. This insight is still expanding and possible therapeutic options for complement modulation are being explored [9,10,11]. Though paroxysmal nocturnal hemoglobinuria (PNH) is not an autoimmune disorder, the entirely complement-dependent pathogenesis and the success of therapeutic complement inhibition in this disease make it possible to learn lessons from PNH that might prove useful in treating AIHA [12]. Diagnostic procedures will not be described as such; comprehensive guidelines for diagnosis can be found elsewhere in the literature [4, 5]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
