Chen S, Eldor A, Barshtein G, Zhang S, Goldfarb A, Rachmilewitz E, Yedgar S
Department of Biochemistry, Hebrew University-Hadassah Medical School, Hadassah University Hospital, Jerusalem, Israel.
Am J Physiol. 1996 Jun;270(6 Pt 2):H1951-6. doi: 10.1152/ajpheart.1996.270.6.H1951.
beta-Thalassemia major (TM), a congenital hemoglobinopathy, is associated with hemodynamic disorders and with structural red blood cell (RBC) anomalies that may indicate impairment of RBC rheological properties. To gain insight into the possible contribution of RBC to the hemodynamic disorders, we studied RBC aggregability, which plays a central role in blood flow, particularly in the microcirculation. RBC aggregate size distribution and morphology of TM RBC were determined using a novel system for image analysis of blood cells in a flow chamber. It was found that the aggregability of RBC of TM patients is markedly enhanced. These cells form large clusters, as opposed to normal rouleaux, and higher shear stress is required to disperse them. The aggregate size of TM RBC is reduced to the normal range after the patients have received a blood transfusion. This study suggests that the hemodynamic disorders observed in TM may be linked to the enhanced RBC aggregability and that improvement of RBC rheological properties may be considered in the treatment of thalassemia.
重型β地中海贫血（TM）是一种先天性血红蛋白病，与血流动力学紊乱以及红细胞（RBC）结构异常有关，这些异常可能表明RBC流变学特性受损。为了深入了解RBC对血流动力学紊乱的可能作用，我们研究了在血流中，尤其是在微循环中起核心作用的RBC聚集性。使用一种新型系统对流动腔室中的血细胞进行图像分析，确定了TM患者RBC的聚集大小分布和形态。结果发现，TM患者的RBC聚集性明显增强。这些细胞形成大的团块，与正常的叠连不同，需要更高的剪切应力才能使其分散。患者接受输血后，TM患者RBC的聚集大小恢复到正常范围。这项研究表明，TM中观察到的血流动力学紊乱可能与RBC聚集性增强有关，并且在治疗地中海贫血时可以考虑改善RBC流变学特性。
