Autoimmune haemolytic anaemia: modern diagnosis and therapy

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Abstract

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.

About the authors

Zhanna A. Kuzminova

Dmitry Rogachev Federal Research Centre of Paediatric Haematology, Oncology and Immunology, Ministry of Health of the Russian Federation

Author for correspondence.
Email: zhanna.kuzminova@fccho-moscow.ru
Russian Federation

Nataliya S. Smetanina

Dmitry Rogachev Federal Research Centre of Paediatric Haematology, Oncology and Immunology, Ministry of Health of the Russian Federation; N.I.Pirogov Russian National Research Medical University

Email: Nataliya.Smetanina@fccho-moscow.ru
Russian Federation

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Copyright (c) 2016 Kuzminova Z.A., Smetanina N.S.

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