巨大血小板综合征的分子机制与临床研究进展
摘要
巨大血小板综合征是一种常染色体隐性遗传性出血性疾病 ,以出血时间延长、血小板减少、血小板巨大为特征 ,其分子基础是编码血小板膜糖蛋白Ib IX V(GPIb IX V)复合物的基因缺陷 ,导致GPIb IX V表达降低或功能异常。患者的出血症状表现为异质性。输注血小板及高剂量因子Ⅶα或重组因子Ⅶα(rFⅦα)是控制出血的有效方法。
出处
《国外医学（生理病理科学与临床分册）》
2004年第2期150-152,共3页
Foreign Medical Sciences(Pathophysiology and Clinical Medicine)
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