Complement Blockade with C1 Esterase Inhibitor in Severe C3d Positive Autoimmune Hemolytic Anemia
نویسندگان
چکیده
منابع مشابه
C1-esterase inhibitor concentrate rescues erythrocytes from complement-mediated destruction in autoimmune hemolytic anemia.
In autoimmune hemolytic anemia (AIHA), autoantibody-mediated complement activation results in C3 deposition on RBCs and possibly the formation of the membrane attack complex cumulating in intravascular hemolysis.1-4 In case of acute signs of tissue hypoxia, life-saving transfusion with RBC units is required. However, recovery of RBC transfusions is inadequate because autoantibodies react with r...
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Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributa...
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ژورنال
عنوان ژورنال: Blood
سال: 2016
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v128.22.4817.4817