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由于血小板膜复合物缺陷导致的血小板病。

Thrombocytopathy due to a defect of the platelet membrane complex.

作者信息

Canizares C, Vivar N, Grijalva J

机构信息

Department of Hematology, Hospital Andrade Marin, IESS, Quito, Ecuador.

出版信息

Acta Haematol. 1990;83(2):99-104. doi: 10.1159/000205178.

Abstract

A new type of primary thrombocytopathy is described. Three main alterations were found: (1) a defect of the aggregation reaction with ADP, epinephrine and collagen and a normal response to ristocetin and arachidonic acid; (2) a moderate deficiency of platelet procoagulant activity, and (3) a combined hypertrophy of the two membrane systems of the platelet--the open canalicular and the dense tubular. The latter defect is shown as an abnormal membrane complex situated on one of the platelet poles. This thrombocytopathy is discussed as a new variety of primary platelet disorder.

摘要

本文描述了一种新型原发性血小板病。发现了三个主要改变:(1)对ADP、肾上腺素和胶原的聚集反应缺陷,而对瑞斯托菌素和花生四烯酸反应正常;(2)血小板促凝活性中度缺乏;(3)血小板的两个膜系统——开放小管系统和致密小管系统联合肥大。后一种缺陷表现为位于血小板一极的异常膜复合物。本文将这种血小板病作为原发性血小板疾病的一种新类型进行了讨论。

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