Quantifying the unique mechanical properties of irreversibly sickled cells in sickle cell disease

Dillon C. Williams , John M. Higgins , David K. Wood
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Abstract

We developed a platform to measure the oxygen-dependent mechanical properties and oxygen saturation of individual irreversibly sickled cells (ISCs). We identified and measured ISCs from a cohort of 10 individuals with sickle cell disease. ISCs were found to have an average shear surface modulus 20 times that of nonsickled cells and a sixth that of red blood cells (RBCs) with detectable hemoglobin polymer. We found that the number of ISCs was significantly reduced at 53 mm Hg oxygen compared with ≥91 mm Hg oxygen, suggesting that these RBCs can still form polymer under hypoxia. We also found that the fraction of ISCs present in a blood sample had a negative correlation with donor fetal hemoglobin (HbF) fraction, suggesting that HbF could play a role in mitigating occurrence of ISCs.
定量镰状细胞病中不可逆镰状细胞的独特力学特性
摘要我们开发了一个测量单个不可逆镰状细胞(ISCs)氧依赖力学特性和氧饱和度的平台。我们从10名镰状细胞病患者的队列中确定并测量了ISCs。发现ISCs的平均剪切表面模量是非镰状细胞的20倍,是具有可检测血红蛋白聚合物的红细胞(rbc)的六分之一。我们发现,与≥91 mm Hg氧相比,53 mm Hg氧条件下ISCs的数量明显减少,这表明这些红细胞在缺氧条件下仍然可以形成聚合物。我们还发现,血液样本中存在的ISCs分数与供体胎儿血红蛋白(HbF)分数呈负相关,这表明HbF可能在减轻ISCs发生方面发挥作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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