Centre de référence des syndromes drépanocytaires majeurs, thalassémies et autres pathologies rare du globule rouge et de l'érythropoïèse, Assistance Publique des Hôpitaux de Marseille, Marseille, France.
Département de médecine interne, Hôpital de la Timone, Assistance Publique des Hôpitaux de Marseille, Marseille, France.
Eur J Haematol. 2023 Mar;110(3):271-279. doi: 10.1111/ejh.13902. Epub 2022 Nov 30.
The vascular endothelium is markedly disrupted in sickle cell disease (SCD) and is the converging cascade of the complex pathophysiologic processes linked to sickle cell vasculopathy. Circulating endothelial activation and/or apoptotic markers may reflect this endothelial activation/damage that contributes to the pathophysiology of the SCD vascular complications.
Plasmatic levels of circulating endothelial cells (CECs), E-selectin, progenitor's endothelial cells (EPCs), and circulating extracellular vesicles (EVs) were evaluated in 50 SCD patients, 16 with vasculopathy. The association between these markers and the occurrence of disease-related microvascular injuries of the eye (retinopathy), kidney (nephropathy), and skin (chronic active ulcers) was explored.
Among the endothelial activation markers studied, only higher plasma levels of E-selectin were found in SCD patients with vasculopathy (p = .015). Increased E-selectin levels were associated with retinopathy (p < .001) but not with nephropathy or leg ulcers. All patients, at steady state, with or without vasculopathy, did not display a high count of CEC and EPC, markers of endothelial injury and repair. We did not show any significant differences in EVs levels between vasculopathy and not vasculopathy SCD patients.
Further studies will be required to determine whether the E-selectin could be used as an early biomarker of retinopathy sickle cell development.
镰状细胞病 (SCD) 患者的血管内皮明显受损，是与镰状细胞血管病相关的复杂病理生理过程的汇聚级联。循环内皮细胞激活和/或凋亡标志物可能反映了这种内皮激活/损伤，有助于 SCD 血管并发症的病理生理。
评估了 50 名 SCD 患者（16 名有血管病）的循环内皮细胞 (CEC)、E-选择素、祖内皮细胞 (EPC) 和循环细胞外囊泡 (EV) 的血浆水平。探讨了这些标志物与眼部（视网膜病变）、肾脏（肾病）和皮肤（慢性活动性溃疡）疾病相关微血管损伤的发生之间的关系。
在所研究的内皮激活标志物中，仅发现血管病 SCD 患者的血浆 E-选择素水平升高（p=0.015）。E-选择素水平升高与视网膜病变相关（p<0.001），但与肾病或腿部溃疡无关。所有处于稳定状态的患者，无论有无血管病，均未显示出 CEC 和 EPC 的高计数，这些标志物代表了内皮损伤和修复。我们没有显示 EVs 水平在血管病和非血管病 SCD 患者之间有任何显著差异。
需要进一步研究以确定 E-选择素是否可作为镰状细胞视网膜病变发展的早期生物标志物。
