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血小板贮存池缺陷:出血时间延长且血小板聚集正常患者的诊断

Platelet storage pool deficiency: diagnosis in patients with prolonged bleeding times and normal platelet aggregation.

作者信息

Israels S J, McNicol A, Robertson C, Gerrard J M

机构信息

Manitoba Institute of Cell Biology, University of Manitoba, Winnipeg, Canada.

出版信息

Br J Haematol. 1990 May;75(1):118-21. doi: 10.1111/j.1365-2141.1990.tb02626.x.

Abstract

We evaluated 46 patients with prolonged bleeding times, and no demonstrable abnormalities of either von Willebrand factor or platelet aggregation, for possible deficiency of platelet storage pool. Studies of ATP release from thrombin-stimulated platelets and enumeration of dense granules in platelet whole mounts were performed in these patients. Seventeen patients (35%) had both decreased ATP release and decreased numbers of dense granules, suggesting the presence of a storage pool defect. Thus, storage pool deficiency may be present in the absence of the classical aggregation abnormalities. Evidence of storage pool deficiency should be considered in all patients with an isolated unexplained prolongation of the bleeding time. The methods used in this study are readily applicable to most clinical laboratories.

摘要

我们评估了46例出血时间延长、血管性血友病因子或血小板聚集无可证实异常的患者,以确定是否存在血小板储存池缺乏。对这些患者进行了凝血酶刺激血小板的ATP释放研究以及血小板整装片中致密颗粒的计数。17例患者(35%)ATP释放减少且致密颗粒数量减少,提示存在储存池缺陷。因此,在无典型聚集异常的情况下可能存在储存池缺乏。对于所有孤立性不明原因出血时间延长的患者,均应考虑存在储存池缺乏的证据。本研究中使用的方法很容易应用于大多数临床实验室。

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