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- W3136342236 endingPage "187" @default.
- W3136342236 startingPage "177" @default.
- W3136342236 abstract "Sickle cell disease (SCD) is a widely spread inherited hemoglobinopathy that includes a group of congenital hemolytic anemias, all characterized by the predominance of sickle hemoglobin (HbS). Its features are anemia, predisposal to bacterial infections and complications such as vaso-occlusive crisis (VOC) or delayed hemolytic transfusion reaction (DHTR), which lead to increased rate of morbidity and mortality even in the era of hydroxyurea. The interaction between sickle cells, neutrophils, platelets or endothelial cells in small vessels results in hemolysis and has been considered the disease's main pathophysiological mechanism. Complement activation has been reported in small cohorts of SCD patients, but the governing mechanism has not been fully elucidated. This will be important to predict the patient group that would benefit from complement inhibition. Until now, eculizumab-mediated complement inhibition has shown beneficial effects in DHTR, with limited reports in patients with VOC. In the meantime, several innovative agents are under clinical development Our state-of-the-art review summarizes current data on 1) complement activation in SCD both in steady state and crisis, 2) underlying mechanisms of complement over-activation for the clinician in the context of SCD, 3) actions of hydroxyurea and new therapeutic approaches including indirect involvement in complement activation, and 4) novel paradigms in complement inhibition." @default.
- W3136342236 created "2021-03-29" @default.
- W3136342236 creator A5017466117 @default.
- W3136342236 creator A5031430901 @default.
- W3136342236 creator A5051463822 @default.
- W3136342236 creator A5061193434 @default.
- W3136342236 creator A5086983982 @default.
- W3136342236 date "2021-03-01" @default.
- W3136342236 modified "2023-10-17" @default.
- W3136342236 title "Complement in Sickle Cell Disease: Are We Ready for Prime Time?" @default.
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