Journal
TRANSFUSION MEDICINE REVIEWS
Volume 33, Issue 4, Pages 225-230Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.tmrv.2019.09.007
Keywords
Complement; Sickle cell disease; Post-transfusion hemolysis
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Transfusion-related hemolysis is classically the result of an interaction between antibodies produced by the recipient and blood group antigens carried by the donor red blood cells. This reaction may be life threatening, especially in sickle cell patients when they develop hyperhemolysis with concomitant accelerated clearance of their own red blood cells. The complement system is a key participant in the pathophysiology of post-transfusion hemolysis. Complement can trigger the hemolytic reaction, amplify the inflammatory response and increase tissue damage. Complement is activated by the classical pathway but may also be activated by the alternative pathway in sickle cell disease. The hemolysis-derived products permanently released by sickle cell patients with chronic hemolytic anemia may affect the potency of complement activation. All the observations in sickle cell patients as well as in vitro experiments and in vivo data in animal models support the conclusion that complement is key disease driver and a promising therapeutic target in the context of transfusion related hemolysis and hyperhemolysis. (C) 2019 Elsevier Inc. All rights reserved.
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