Treatment of various forms of autoimmune hemolytic anemia and monitoring hemolysis activity

Autoimmune haemolytic anaemia (AIHA) is a rare disease characterized by formation of red blood cell autoantibodies. A precise classification of AIHA types is very important. There are warm (w-AIHA) and cold (c-AIHA) AIHA with cold haemolysins, and drug-induced AIHA. Each type has different serological characteristics of autoantibodies, which determines the mechanism of red blood cell destruction. Involvement of the complement plays a certain but limited role in w-AIHA, whereas c-AIHA is entirely complement-dependent. In this connection, therapies for different kinds of AIHA also differ. Thus, in w-AIHA the first-line drugs are glucocorticosteroids, while in c-AIHA their efficacy is disputable. In relapses and refractory haemolyses, administration of rituximab and splenectomy are recommended as the second-line therapy for w-AIHA. In c-AIHA, early administration of rituximab is recommended as the first-line therapy. The use of other immunosuppressive drugs (azathioprine, cyclophosphamide, cyclospirin A, mofetil mycophenolate) as alternative therapy is possible but their effectiveness is significantly lower.