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一种与出血倾向相关的未分类血小板功能障碍。

An unclassified platelet function disorder associated with bleeding tendency.

作者信息

Endo Y, Mamiya S, Niitsu H, Iwamoto K, Hamanaka S, Miura A B

出版信息

Tohoku J Exp Med. 1987 Apr;151(4):387-94. doi: 10.1620/tjem.151.387.

DOI:10.1620/tjem.151.387
PMID:2956728
Abstract

A 7-year-old boy with a history of bleeding tendency showed a prolongation of bleeding time and a decrease in platelet adhesiveness. The platelets of the patient, however, had a normal reaction to ADP, collagen, epinephrine, arachidonic acid, bovine fibrinogen, ristocetin, A-23187 and thrombin-induced aggregation, and their shape was determined by electron microscopy to be normal. Therefore, this disorder could not be thought to belong to any known platelet dysfunction. On the other hand, an increase in clot retraction, a reversal of ATP/ADP, a decrease in beta-thromboglobulin and platelet factor 4 in the platelets, and an elevation of plasma levels of released beta-thromboglobulin from the platelets were observed in the patient. We don't know any cases with such an association of hypo- and hyperfunction of platelets.

摘要

一名有出血倾向病史的7岁男孩,出血时间延长,血小板黏附性降低。然而,该患者的血小板对二磷酸腺苷(ADP)、胶原、肾上腺素、花生四烯酸、牛纤维蛋白原、瑞斯托霉素、A - 23187和凝血酶诱导的聚集反应正常,通过电子显微镜观察其形态也正常。因此,这种病症不能被认为属于任何已知的血小板功能障碍。另一方面,在该患者中观察到凝块回缩增加、三磷酸腺苷(ATP)/二磷酸腺苷(ADP)比值逆转、血小板中β - 血小板球蛋白和血小板因子4减少以及血小板释放的β - 血小板球蛋白血浆水平升高。我们不知道有任何血小板功能减退和亢进相关联的病例。

相似文献

1
An unclassified platelet function disorder associated with bleeding tendency.一种与出血倾向相关的未分类血小板功能障碍。
Tohoku J Exp Med. 1987 Apr;151(4):387-94. doi: 10.1620/tjem.151.387.
2
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