临床儿科杂志 ›› 2015, Vol. 33 ›› Issue (5): 486-.doi: 10.3969 j.issn.1000-3606.2015.05.022

• 文献综述 • 上一篇    下一篇

遗传性血小板无力症发病机制及治疗进展

高敏综述,苏庸春审校   

  1. 重庆医科大学附属儿童医院血液肿瘤科 儿童发育疾病研究教育部重点实验室 儿科学重庆市重点实验室( 重庆 400014)
  • 收稿日期:2015-05-15 出版日期:2015-05-15 发布日期:2015-05-15

An update on pathogenesis and treatment of Glanzmann thrombasthenia

Reviewer:GAO Min, Reviser:SU Yongchun   

  1. Hematology Department of Children’s Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing,Chongqing 400014, China
  • Received:2015-05-15 Online:2015-05-15 Published:2015-05-15

摘要: 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。

Abstract:  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.