Autoimmune hemolytic anemia

Česká verzia

Authors: Jaroslav Čermák ^1,2; Martin Písačka ^1,2
Authors place of work: Ústav klinické a experimentální hematologie 1. LF UK, Praha ¹; Ústav hematologie a krevní transfuze, Praha ²
Published in the journal: Vnitř Lék 2018; 64(5): 514-519
Category:

Summary

Autoimmune hemolytic anemia (AIHA) is caused by auto-antibodies directed against self red blood cell (RBC) surface antigens. A consequence may be an intravascular hemolysis mediated by activated complement or extravascular hemolysis caused by destruction of complex of RBC with autoantibody in spleen and liver. The basic classification subdivides AIHA in primary/idiopathic and secondary with known underlying disease. A classification according to the thermal range of antibody recognizes warm AIHA, cold aglutinin disease (CAD), mixed AIHA and paroxysmal cold hemoglobinuria. Pathogenesis of AIHA consists of a defective antigen presentation to immunocompetent cells, insufficient process of T-lymphocyte tolerance to autoantigens and induction of autoantibody production by B-lymphocytes. For the diagnosis of AIHA are essential direct and indirect antiglobulin tests. The first-line therapy for warm AIHA is still administration of corticosteroids. For non-responding patients, second-line treatment includes rituximab or splenectomy. Combination of other immunosuppressive drugs represents a third-line treatment for resistant/relapsing patients. Rituximab is a treatment of choice for patients with CAD.

Key words:

anemia hemolytic – autoimmunity – corticosteroids – diagnosis – pathogenesis – rituximab – splenectomy – treatment

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