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Complement involvement in sickle cell disease
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DOI：10.1016/j.lpm.2023.104205.png)
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Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of SCD, contributing to the vascular complications and tissue damage. This review is centered on exploring the role of the inflammatory complement system in the pathophysiology of SCD. Our goal is to offer a comprehensive summary of the existing evidence regarding complement activation in patients with SCD, encompassing both steady-state conditions and episodes of vaso-occlusive events. Additionally, we will discuss the proposed mechanisms by which the complement system may contribute to tissue injury in this pathology. Finally, we will provide an overview of the available evidence concerning the effectiveness of therapeutic interventions aimed at blocking the complement system in the context of SCD and discuss the perspective of complement inhibition. (c) 2023 Elsevier Masson SAS. All rights reserved.
Keyword:
MEDIATED DEPOSITION
ERYTHROCYTES
HEMOGLOBIN
HEMOLYSIS
FRAGMENTS
THERAPY
MODEL
期刊
P
IF:
3.4
论文数:
4.5K
被引数:
2.3K
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Heme Drives Susceptibility of Glomerular Endothelium to Complement Overactivation Due to Inefficient Upregulation of Heme Oxygenase-1由于血红素Oxygenase-1的低效上调，血红素驱动肾小球内皮对补体过度激活的敏感性
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OAAI分享收藏In vitro evidence of complement activation in patients with sickle cell disease.png)
HAEMATOLOGICAOAAI
HAEMATOLOGICAIF7.9
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