Clinical manifestations and erythrocyte adhesion to endothelium in sickle cell syndrome

Clinical manifestations and erythrocyte adhesion to endothelium in sickle cell syndrome
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镰状细胞综合征的临床表现和红细胞内皮粘附

DOI:
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发表时间:
1985
期刊:
American journal of hematology/oncology
影响因子:
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通讯作者:
J. Caen
J. Caen
中科院分区:
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文献类型:
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作者:
J. Wautier;F. Galactéros;M. Wautier;D. Pintigny;Y. Beuzard;J. Rosa;J. Caen

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疼痛性血管闭塞发作是镰状细胞病最突出的病理特征之一。除了形状异常和变形性差外,还报告了红细胞与内皮细胞的粘附增加。在几个场合,我们研究了30例混合型镰状细胞综合征患者红细胞的粘附,以评估临床条件的影响。镰状病人红细胞粘附率明显高于对照组(P < 0.01)。此外,当患者处于危象时,粘附性显著更高(p < 0.01),并且在具有炎症状况的患者中观察到所有粘附性的最高值。为了研究有限的红细胞群可能导致红细胞粘附增加的可能性,我们测量了不同洗涤液中的HbS浓度,发现最后洗涤液中的HbS浓度高于第一次洗涤液。用[3H]亮氨酸标记能够合成蛋白质的镰状红细胞(年轻红细胞)。[3H]亮氨酸标记的红细胞与内皮细胞的粘附性高于同一患者的51Cr标记的红细胞。另一方面,通过密度梯度分离的最致密的镰状红细胞比浅红色细胞粘附的程度更大。这种明显的差异可以部分解释为在通过stractan梯度分离的镰状红细胞的致密部分中存在[3H]亮氨酸标记的红细胞。
Painful vasocclusive episodes are one of the most prominent pathological features of sickle cell disease. In addition to abnormal shape and poor deformability, increased adhesion of red cells to endothelium has been reported. On several occasions, we have studied the adhesion of erythrocytes from 30 patients with mixed sickle cell syndromes to evaluate the influence of clinical conditions. The percentage of erythrocytes adhering was significantly higher when erythrocytes from sickle patients were compared with controls (p < 0.01). Furthermore, adhesion was significantly higher when the patients were in crises (p < 0.01), and the highest values of all were observed in patients with inflammatory conditions. To investigate the possibility that a limited population of red cells could be responsible for the increase in red cell adhesion, we have measured the HbS concentration in the different washes and found that the HbS concentration was higher in the last washes compared to the first washes. Sickle red cells capable of protein synthesis (young red cells) were labelled with [3H] leucine. The adhesion to endothelial cells of [3H] leucine‐labelled red cells was higher than that of the 51Cr‐labelled red cells from the same patient. On the other hand, the most dense sickle red cells separated by density gradient adhered to a greater extent than the light red cells. This apparent discrepancy could be partly explained by the presence of [3H] leucine‐labelled red cells in the dense fractions of sickle red cells separated by stractan gradient.