Abstract：
Sickle cell anemia is a genetic disorder that affects hemoglobin leading to the production of an abnormal hemoglobin, called HbS. HbS has the property to polymerize under deoxygenated conditions, causing a mechanical distortion of red blood cells; a phenomenon called sickling. These sickle red blood cells are more fragile and rigid, leading to chronic hemolytic anemia and painful vaso-occlusive crises, as well as chronic vascular complications that can affect many organs. The abnormal functional properties of these sickle red blood cells are responsible for a wide range of clinical expression of the disease. HbS polymerization can be influenced by many factors, such as the hydration state of the red blood cells or the affinity of hemoglobin for oxygen. Moreover, the rheological characteristics of red blood cells, including their deformability and aggregation properties, are associated with specific clinical phenotypes. The pro-inflammatory and pro-oxidant state, as well as the repeated polymerization of HbS, accelerate the senescence of sickle red blood cells, promoting the release of microparticles and contributing to vascular dysfunction. Patients\' red blood cells also have molecular characteristics that promote their adhesion to the endothelium and other circulating cells, contributing to the onset of vascular complications. Massive intravascular hemolysis, due to increased erythrocyte fragility, is also responsible for chronic vascular complications. These different alterations are privileged therapeutic targets, leading to the emergence of new specific treatments. © 2023 Société nationale française de médecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.
摘要：
镰状细胞性贫血是一种影响血红蛋白导致异常血红蛋白产生的遗传性疾病，叫做HBS。HbS具有在脱氧条件下聚合的性质，引起红细胞的机械变形；一种叫做镰状的现象。这些镰状红细胞更脆弱和僵硬，导致慢性溶血性贫血和痛苦的血管闭塞危机，以及可能影响许多器官的慢性血管并发症。这些镰状红细胞的异常功能特性是该疾病广泛临床表现的原因。HbS聚合受多种因素影响,例如红细胞的水合状态或血红蛋白对氧的亲和力。此外，红细胞的流变特性，包括它们的变形性和聚集性，与特定的临床表型相关。促炎和促氧化状态，以及HbS的重复聚合，加速镰状红细胞的衰老，促进微粒的释放并导致血管功能障碍。患者的红细胞还具有促进其与内皮和其他循环细胞粘附的分子特征，导致血管并发症的发生。大量血管内溶血，由于红细胞脆性增加，也是慢性血管并发症的原因。这些不同的改变是特权治疗目标，导致新的特定治疗方法的出现。©2023法国国家石油公司（SNFMI）。由ElsevierMassonSAS发布。保留所有权利。
