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Complement activation during intravascular hemolysis: Implication for sickle cell disease and hemolytic transfusion
N S Merle1, I Boudhabhay1, J Leon1
1Centre de recherche des cordeliers, Inserm, Sorbonne université, USPC, Université Paris Descartes, Université Paris Diderot, 75006 Paris, France.
Summary
Intravascular hemolysis, common in sickle cell disease (SCD), involves red blood cell (RBC) breakdown products that damage tissues. The complement system amplifies this damage, but its blockade shows promise for treating related conditions like delayed hemolytic transfusion reactions (DHTR).
Area of Science:
- Immunology
- Hematology
- Vascular Biology
Background:
- Intravascular hemolysis releases damaging red blood cell (RBC) products, contributing to diseases like sickle cell disease (SCD).
- The complement system, part of innate immunity, is activated by hemolysis products and hypoxia, amplifying cell and tissue damage.
- This review examines the complement system's role in hemolysis-associated injuries in SCD and delayed hemolytic transfusion reactions (DHTR).
Purpose of the Study:
- To review the involvement of the complement system in hemolysis and related injuries in SCD and DHTR.
- To summarize evidence of complement activation biomarkers in SCD patients.
- To discuss mechanisms of complement activation in DHTR and therapeutic blockade efficacy.
Main Methods:
- Review of existing literature on intravascular hemolysis, SCD, DHTR, and the complement system.
- Analysis of evidence for complement activation biomarkers in SCD.
- Examination of mechanisms of complement activation (classical and alternative pathways) in DHTR.
- Assessment of data on therapeutic complement blockade for DHTR.
Main Results:
- Biomarkers of complement activation are present in SCD patients.
- Both antibody-dependent classical and heme-dependent alternative complement pathways are implicated in DHTR.
- Evidence suggests therapeutic complement blockade is effective for DHTR.
Conclusions:
- Complement system activation significantly contributes to hemolysis-mediated injuries in SCD and DHTR.
- Therapeutic complement blockade shows promise for treating DHTR.
- Further prospective studies are needed to establish complement inhibitors like Eculizumab for DHTR and potentially SCD.
Keywords:
ComplementComplémentDrépanocytoseHemolytic transfusion reactionHémolyse intravasculaireHémolyse post-transfusionnelleIntravascular hemolysisSickle cell diseaseMore Related Videos
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