Sadaf Alina, Seu Katie G, Thaman Elizabeth, Fessler Rose, Konstantinidis Diamantis G, Bonar Holly A, Korpik Jennifer, Ware Russell E, McGann Patrick T, Quinn Charles T, Kalfa Theodosia A
Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Front Physiol. 2021 Mar 25;12:636609. doi: 10.3389/fphys.2021.636609. eCollection 2021.
Sickle cell anemia (SCA) is a hereditary hemoglobinopathy with a variable phenotype. There is no single biomarker that adequately predicts disease severity and can be used to monitor treatment response in patients in clinical trials and clinical care. The use of clinical outcomes, such as vaso-occlusive crises (VOC), requires long and expensive studies, sometimes with inconclusive results. To address these limitations, there are several biomarkers under study to improve the ability to predict complications and assess treatment response in both clinical and research settings. Oxygen gradient ektacytometry, also called as oxygenscan, is an assay that measures the effects of deoxygenation and reoxygenation on red blood cell (RBC) deformability and is gaining popularity in SCA research, because it captures the dynamic sickling capacity of a patient's RBCs as they are subjected to an oxygen gradient under steady shear stress. We describe here the oxygenscan methodology and evaluate the correlation between oxygenscan parameters and more well-known biomarkers of SCA such as fetal hemoglobin (HbF), F-cells, and dense red blood cells (DRBCs). Our data indicate that the oxygenscan curve is affected by all these parameters and the result incorporates the effects of %HbF, %F-cells, RBC hydration, and RBC membrane deformability.
镰状细胞贫血(SCA)是一种具有可变表型的遗传性血红蛋白病。没有单一的生物标志物能够充分预测疾病严重程度,也无法用于监测临床试验和临床护理中患者的治疗反应。使用临床结局,如血管闭塞性危机(VOC),需要进行长期且昂贵的研究,有时结果并不确定。为解决这些局限性,目前有几种生物标志物正在研究中,以提高在临床和研究环境中预测并发症及评估治疗反应的能力。氧梯度红细胞变形性测定法,也称为氧扫描,是一种测量脱氧和复氧对红细胞(RBC)变形性影响的检测方法,在SCA研究中越来越受欢迎,因为它能捕捉患者红细胞在稳定剪切应力下受到氧梯度作用时的动态镰变能力。我们在此描述氧扫描方法,并评估氧扫描参数与SCA更知名的生物标志物(如胎儿血红蛋白(HbF)、F细胞和致密红细胞(DRBC))之间的相关性。我们的数据表明,氧扫描曲线受所有这些参数的影响,其结果包含了HbF百分比、F细胞百分比、RBC水合作用和RBC膜变形性的影响。