Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children16310.24287/1726-1708-2016-15-3-5-11HEMATOLOGYГЕМАТОЛОГИЯAutoimmune haemolytic anaemia: modern diagnosis and therapyАутоиммунная гемолитическая анемия: современная диагностика и терапияKuzminovaZhanna A.КузьминоваЖанна Андреевна
Russian Federation
zhanna.kuzminova@fccho-moscow.ruSmetaninaNataliya S.СметанинаНаталия Сергеевна
Russian Federation
Nataliya.Smetanina@fccho-moscow.ruDmitry Rogachev Federal Research Centre of Paediatric Haematology, Oncology and Immunology, Ministry of Health of the Russian FederationФедеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава РоссииN.I.Pirogov Russian National Research Medical UniversityРоссийский национальный исследовательский медицинский университет им. Н.И.Пирогова Минздрава России1909201615351119092018Copyright ©; 2016, «D. Rogachev NMRCPHOI»Copyright ©; 2016, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2016«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/163

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.

аутоиммунная гемолитическая анемияпароксизмальная холодовая гемоглобинуриятепловая аутоиммунная гемолитическая анемияхолодовая аутоиммунная гемолитическая анемияautoimmune haemolytic anaemiaparoxysmal cold haemoglobinuriawarm autoimmune haemolytic anaemiacold autoimmune haemolytic anaemiaMarcus N, Attias D, Tamary H. Autoimmune hemolytic anemia: current understanding of pathophysiology. Hematology Education: the education program for the annual congress of the European Hematology Association. 2014; 8:331-8.Berentsen S, Sundic T. Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy. Bio Med Res Int. 2015;2015:363278.Petz LD, Garatty G. Classification and clinical characteristics of autoimmune hemolytic anemias (chapter 3). In: Immune hemolytic anemias. Petz LD, Garatty G (eds). 2nd edition. Philadelphia: Churchill Livingstone, 2004;61 -131.Li Z, Shao Z, Xu Y, Shen L, Chen G, Zhang Y, et al. Subclasses of warm autoantibody IgG in patients with autoimmune hemolytic anemia and their clinical implications. Chin Med J (Engl). 1999;112(9):805-8.Segel GB, Lichtman MA. Direct antiglobulin (“Coombs”) test-negative autoimmune hemolytic anemia: a review. Blood Cell Mol Dis. 2014;52(4):152-60.Zenella A, Barcellini W. Treatment of autoimmune hemolytic anemias. Hematology Education: The education program for the annual congress of the European hematology association. 2014;8:339-48.Цветаева НВ, Никулина ОФ. Клинические рекомендации по диагностике и лечению аутоиммунных гемолитических анемий. Под ред. Савченко ВГ. Национальное гематологическое общество. 2014. Режим доступа: http:// zdravalt.ru/jdownloads/protokol/gemat_obshch_aiga.pdfMeyer O, Stahl D, Beckhove P, Huhn D, Salama A. Pulsed high-dose dexamethasone in chronic autoimmune haemolytic anaemia of warm type. Br J Haematol. 1997;98(4):860-2.Dierickx D, Verhoef G, Van Hoof A, Mineur P, Roest A, Triffet A, et al. Rituximab in auto-immune haemolytic anaemia and immune thrombocytopenic purpura: a Belgian retrospective multicentric study. J Intern Med. 2009;266(5):484-91.Barcelli W, Zanella A. Rituximab therapy for autoimmune haematological diseases. Eur J Intern Med. 2011;22(3):220-9.Birgens H, Frederiksen H, Hasselbalch HC, Rasmussen IH, Nielsen OJ, Kjeldsen L, et al. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. Br J Haematol. 2013;163(3):393-9.Barcellini W, Zaja F, Zaninoni A, Imperiali FG, Battista ML, Di Bona E, et al. Low-dose rituximab in adult patients with idiopathic autoimmune hemolytic anemia: clinical efficacy and biological studies. Blood. 2012;119(16):3691-7.Jaime-Pérez JC, Rodrlguez-Martlnez M, Gömez-de-Leön A, Tarln-Arzaga L, Gömez-Almaguer D. Current approaches for the treatment of autoimmune hemolytic anemia. Arch Immunol Ther Exp (Warsz). 2013;61(5):385-95.Newland A, Provan D, Myint S. Preventing severe infection after splenectomy. BMJ. 2005;331(7514):417-8.Liu H, Shao Z, Jing L. The effectiveness of cyclosporin A in the treatment of autoimmune hemolytic anemia and Evans syndrome. Zhonghua Xue Ye Xue Za Zhi. 2001;22(11):581-3.Rao VK, Dugan F, Dale JK, Davis J, Tretler J, Hurley JK, et al. Use of mycophenolate mofetil for chronic, refractory immune cytopenias in children with autoimmune lymphoproliferative syndrome. Br J Haematol. 2005;129(4):534-8.O’Connell N, Goodyer M, Gleeson M, Storey L, Williams M, Cotter M, et al. Successful treatment with rituximab and mycophenolate mofetil of refractory autoimmune hemolytic anemia post-hematopoietic stem cell transplant for dyskeratosis congenital due to TINF2 mutation. Pediatr Transplant. 2014;18(1):E22-4.Zeerleder S. Autoimmune haemolytic anaemia - a practical guide to cope with a diagnostic and therapeutic challenge. Neth J Med. 2011;69(4):177-84.Lechner L, Jäger U. How I treat autoimmune hemolytic anemias in adults. Blood. 2010;116(11):1831 -8.Salama A, Berghöfer H, Mueller-Eckhardt C. Red blood cell transfusion in warmtype autoimmune haemolytic anaemia. Lancet. 1992;340(8834-8835):1515-7.Arbach O, Funck R, Seibt F, Salama A. Erythropoietin may improve anemia in patients with autoimmune hemolytic anemia associated with reticulocytopenia. Transfus Med Hemother. 2012;39(3):221-3.Berentsen S, Ulvestad E, Langholm R, Beiske K, Hjorth-Hansen H, Ghanima W, et al. Primary chronic cold agglutinin disease: a population based clinical study of 86 patients. Haematologica. 2006;91(4):460-6.Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood. 2013; 122(7):1114-21.Berentsen S. Therapy for chronic cold agglutinin disease: perspective for further improvements. Blood Transfus. 2013;11(2):167-8.Gertz MA. Management of cold haemolytic syndrome. Br J Haematol. 2007; 138(4):422-9.Carson KR, Beckwith LG, Mehta J. Successful treatment of IgM-meduatd autoimmune hemolytic anemia with bortezomib. Blood. 2010;115(4):915.Gueli A, Gottardi D, Hu H, Ricca I, de Crescenzo A, Tarella C. Efficacy of rituximabbendamustine in cold agglutinin haemolytic anaemia refractory to previous chemo-immunotherapy: a case report. Blood Transfus. 2013;11(2):311-4.Gömez-Almaguer D, Solano-Genesta M, Tarln-Arzaga L, Herrera-Garza JL, Cantû-Rodrlguez OG, Gutiérrez-Aguirre CH, et al. Low-dose rituximab and alemtuzumab combination therapy for patients with steroid-refractory autoimmune cytopenias. Blood. 2010;116(23):4783-5.Ziman A, Hsi R, Goldfinger D. Transfusion Medicine Illustrated: DonathLandsteiner antibody-associated hemolytic anemia after Haemophilus influenzae infection in child. Transfusion. 2004;44(8):1127-8.