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血小板无力症患者及杂合子血小板中的糖蛋白IIb-IIIa复合物。

Glycoprotein IIb-IIIa complex in platelets of patients and heterozygotes of Glanzmann's thrombasthenia.

作者信息

Herrmann F H, Meyer M, Gogstad G O, Solum N O

出版信息

Thromb Res. 1983 Dec 15;32(6):615-22. doi: 10.1016/0049-3848(83)90063-4.

Abstract

The concentration of the glycoprotein (GP) IIb-IIIa complex in thrombasthenic platelets of 8 patients of 6 families has been estimated. In the thrombasthenic platelets of 3 patients this complex is absent (thrombasthenia type I and subtype I). In 2 patients only traces are detectable and in 3 patients GP IIb-IIIa complex is strongly reduced (less than 5%). On the basis of the haemostatic data as well as the content of GP IIb-IIIa complex and platelet fibrinogen the classification of these types as subtypes of thrombasthenia type II is discussed. The diagnostic applicability of GP IIb-IIIa complex determination for heterozygote detection in types of thrombasthenia with absent or extremely reduced GP IIb-IIIa complex is shown.

摘要

对6个家族的8例患者血小板无力症血小板中糖蛋白(GP)IIb-IIIa复合物的浓度进行了估算。3例患者的血小板无力症血小板中不存在这种复合物(I型和I亚型血小板无力症)。2例患者仅可检测到微量,3例患者的GP IIb-IIIa复合物显著减少(低于5%)。基于止血数据以及GP IIb-IIIa复合物和血小板纤维蛋白原的含量,讨论了将这些类型归类为II型血小板无力症亚型的情况。显示了GP IIb-IIIa复合物测定在检测GP IIb-IIIa复合物缺失或极度减少的血小板无力症类型杂合子时的诊断适用性。

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