Martorana Maria Cristina, Mojoli Giorgio, Cianciulli Paolo, Tarzia Anna, Mannella Emilio, Caprari Patrizia
Centro Aziendale Produzione Emocomponenti (CAPE), Azienda Ospedaliera San Camillo-Forlanini, Via Ramazzini,15 00151 Rome, Italy.
Ann Ist Super Sanita. 2007;43(2):164-70.
The maintenance of erythrocyte shape and membrane integrity is bound to the modification of deformability and/or permeability. Usually, this features are not investigated with normal laboratory tests. The membrane stiffness, the cell geometry, and the viscoelasticity components are influencing factors on survival and functionality of the erythrocytes. Only few studies have analyzed the viscoelastic characteristics of red blood cells, even less are the studies on patients affected by sickle cell disease (SCD), a pathology characterized by acute and chronic impairment of cell flexibility due to the formation of intracellular sickle haemoglobin (Hb S) polymers. A critical point of SCD is represented by the rheologic alterations of sickle cells determined by the transition from sol to gel of haemoglobin producing a dramatic change in cell viscosity and viscoelastic properties. We have investigated the behaviour of the blood in SCD, from an original rheological point of view, by evaluating the viscoelastic properties of sickle cells in oscillating harmonic sinusoidal mode. A comparison between patients with different severity of the disease, with transfusion dependence (TD) or without transfusion dependence (NTD), has been carried out. This study has confirmed the rheologic impairment of SC blood. The TD patients showed a minor heterogeneneity of rheologic behaviour in comparison with NTD patients, because of the normalizing effect of transfusion. The analysis of viscoelastic properties might be an additional useful tool for monitoring transfusional and pharmacological treatments.
红细胞形状和膜完整性的维持与可变形性和/或通透性的改变密切相关。通常，这些特征无法通过常规实验室检测进行研究。膜硬度、细胞几何形状和粘弹性成分是影响红细胞存活和功能的因素。仅有少数研究分析了红细胞的粘弹性特征，而针对镰状细胞病（SCD）患者的研究更少，SCD是一种由于细胞内镰状血红蛋白（Hb S）聚合物形成导致细胞柔韧性急性和慢性受损的疾病。SCD的一个关键点是镰状细胞的流变学改变，这是由血红蛋白从溶胶向凝胶的转变所决定的，会导致细胞粘度和粘弹性特性发生显著变化。我们从一个全新的流变学角度，通过评估镰状细胞在振荡谐波正弦模式下的粘弹性特性，研究了SCD患者血液的行为。我们对不同疾病严重程度、有输血依赖（TD）或无输血依赖（NTD）的患者进行了比较。这项研究证实了SCD患者血液的流变学损伤。由于输血的归一化作用，TD患者与NTD患者相比，流变学行为的异质性较小。粘弹性特性分析可能是监测输血和药物治疗的另一个有用工具。
