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2015 ; 100
(11
): 1388-95
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Complement inhibitors to treat IgM-mediated autoimmune hemolysis
#MMPMID26521297
Wouters D
; Zeerleder S
Haematologica
2015[Nov]; 100
(11
): 1388-95
PMID26521297
show ga
Complement activation in autoimmune hemolytic anemia may exacerbate extravascular
hemolysis and may occasionally result in intravascular hemolysis. IgM
autoantibodies as characteristically found in cold autoantibody autoimmune
hemolytic anemia, in cold agglutinin disease but also in a considerable
percentage of patients with warm autoantibodies are very likely to activate
complement in vivo. Therapy of IgM-mediated autoimmune hemolytic anemia mainly
aims to decrease autoantibody production. However, most of these treatments
require time to become effective and will not stop immediate ongoing
complement-mediated hemolysis nor prevent hemolysis of transfused red blood
cells. Therefore pharmacological inhibition of the complement system might be a
suitable approach to halt or at least attenuate ongoing hemolysis and improve the
recovery of red blood cell transfusion in autoimmune hemolytic anemia. In recent
years, several complement inhibitors have become available in the clinic, some of
them with proven efficacy in autoimmune hemolytic anemia. In the present review,
we give a short introduction on the pathogenesis of autoimmune hemolytic anemia,
followed by an overview on the complement system with a special focus on its
regulation. Finally, we will discuss complement inhibitors with regard to their
potential efficacy to halt or attenuate hemolysis in complement-mediated
autoimmune hemolytic anemia.
|*Autoantibodies/blood/immunology
[MESH]
|*Complement System Proteins/immunology/metabolism
[MESH]