遗传性血小板无力症是一种遗传性血小板功能障碍性疾病，由于血小板膜糖蛋白GPⅡb/Ⅲa数量或结构异常，导致血小板对多种诱聚剂反应不良。临床表现为自幼反复发生的自发性出血，且常伴终生。本病属罕见遗传性疾病，尚无统一的根治性治疗措施。文章对遗传性血小板无力症的发病机制及治疗进展作一综述。

　Glanzmann thrombasthenia (GT) is an inherited disease of platelet function disorders characterized by mucocutaneous bleeding due to platelets failed to aggregate in response to physiologic stimuli. GT is a rare inherited disease and caused by quantitative or qualitative deficiencies of an integrin receptor GP Ⅱb/Ⅲa for adhesive proteins. There is no unified treatment strategy available so far for GT. This review summarizes the update of pathogenesis treatment progresses in GT.