Sickle cell disease as a vascular disorder.
Sickle cell disease as a vascular disorder.
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镰状细胞病是一种血管疾病。
DOI：
10.1080/17474086.2020.1758555
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发表时间：
2020
影响因子：
2.8
通讯作者：
Ofori-Acquah,SolomonF
中科院分区：
文献类型：
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作者：
Ofori-Acquah,SolomonF
简介在镰状细胞病（SCD）中，血红蛋白S（HbS）红细胞（RBC）具有变形和不灵活的特征。它们通常在循环中分解，表现出与内皮细胞和活化的中性粒细胞和血小板的粘附性增加，增加了微循环阻塞的风险。SCD分为两种亚表型：高溶血性，与阴茎异常勃起、腿部溃疡、肺动脉高压和中风相关，以及高血红蛋白/粘度，其可促进血管闭塞相关疼痛、急性胸部综合征和骨坏死。溶血可能引发血管闭塞，导致血管并发症。靶向P-选择素，高溶血和血管闭塞之间的关键介质，可能有利于血管和血管闭塞相关的并发症。使用检索词“镰状细胞病”、“血管闭塞性危象”和“选择素”，对2000年1月1日至2019年1月1日期间PubMed中关于SCD和血管闭塞性危象（VOC）主题的英文文章进行了综述。专家意见除了针对P-选择素，其他策略，以对付挥发性有机化合物和红细胞镰状化正在进行中。这些包括血小板抑制以对抗VOC期间的聚集、细胞间粘附和血栓形成;基因治疗以纠正β-珠蛋白基因中的纯合错义突变，引起HbS聚合; L-谷氨酰胺，可能降低镰状红细胞中的氧化应激;以及胎儿血红蛋白诱导剂。
IntroductionIn sickle cell disease (SCD), hemoglobin S (HbS) red blood cells (RBCs) are characteristically deformed and inflexible. Often breaking down in the circulation, they exhibit increased adhesive properties with the endothelium and activated neutrophils and platelets, increasing the risk of occlusion of the microcirculation. SCD is categorized into two sub-phenotypes: hyperhemolytic, associated with priapism, leg ulcers, pulmonary hypertension, and stroke, and high hemoglobin/viscosity, which may promote vaso-occlusion-associated pain, acute chest syndrome, and osteonecrosis.Areas coveredThe sub-phenotypes are not completely distinct. Hemolysis may trigger vaso-occlusion, contributing to vascular complications. Targeting P-selectin, a key mediator of cross-talk between hyperhemolysis and vaso-occlusion, may be beneficial for vascular and vaso-occlusion-associated complications. English-language articles from PubMed on the topic of SCD and vaso-occlusive crises (VOCs) were reviewed from 1 January 2000 to 1 January 2019 using the search terms ‘sickle cell disease,’ ‘vaso-occlusive crises,’ and ‘selectin.’Expert opinionBesides targeting P-selectin, other strategies to counter VOCs and RBC sickling are being pursued. These include platelet inhibition to counter aggregation, intercellular adhesion, and thrombosis during VOCs; gene therapy to correct the homozygous missense mutation in the β-globin gene, causing polymerization of HbS; L-glutamine, possibly reducing oxidative stress in sickled RBCs; and fetal hemoglobin inducers.