文献综述

遗传性血小板无力症发病机制及治疗进展

  • 高敏
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  • 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

  网络出版日期: 2015-05-15

An update on pathogenesis and treatment of Glanzmann thrombasthenia

  • GAO Min
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  • 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 date: 2015-05-15

  Online 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。

本文引用格式

高敏 . 遗传性血小板无力症发病机制及治疗进展[J]. 临床儿科杂志, 2015 , 33(5) : 486 . DOI: 10.3969 j.issn.1000-3606.2015.05.022

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.
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