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一名嗜酸性粒细胞白血病患者的血小板缺陷:瑞斯托霉素诱导的血小板聚集缺失与血小板唾液酸含量降低相关。

A platelet defect in a patient with eosinophilic leukaemia: absent ristocetin-induced platelet aggregation associated with a reduced platelet sialic acid content.

作者信息

Wautier J L, Souchon H, Dupuis D, Caen J P, Nurden A T

出版信息

Scand J Haematol. 1979 Mar;22(3):267-76. doi: 10.1111/j.1600-0609.1979.tb02808.x.

Abstract

Platelets from a patient with eosinophilic leukaemia were not aggregated by ristocetin. The defect was not corrected by normal human plasma and was due to a platelet abnormality. The patient's platelets also showed a diminished sensitivity to aggregation by bovine factor VIIIVWF. The defect was not associated with a prolonged bleeding time. No abnormalities were detected in ADP, collagen or thrombin-induced platelet aggregation. Biochemical studies showed that the platelets were deficient in sialic acid. This deficiency was associated with a reduced staining for glycoprotein I following SDS-polyacrylamide gel electrophoresis. The results suggest an acquired platelet surface abnormality.

摘要

一名嗜酸性粒细胞白血病患者的血小板不会被瑞斯托霉素凝集。正常人血浆无法纠正该缺陷,其原因是血小板异常。该患者的血小板对牛因子VIIIVWF诱导的凝集反应敏感性也降低。该缺陷与出血时间延长无关。在ADP、胶原或凝血酶诱导的血小板凝集过程中未检测到异常。生化研究表明,这些血小板缺乏唾液酸。这种缺乏与SDS-聚丙烯酰胺凝胶电泳后糖蛋白I染色减少有关。结果提示存在一种获得性血小板表面异常。

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