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魁北克血小板紊乱症

Quebec platelet disorder.

作者信息

Abbasi Anwar Hussain, Shaikh Quratulain, Hussain Syed Ather

机构信息

Department of Medicine, The Aga Khan University Hospital, Karachi.

出版信息

J Coll Physicians Surg Pak. 2010 Aug;20(8):549-50.

Abstract

A case of Quebec platelet disorder is hereby reported. A 33 years old woman presented with history of epistaxis and gum bleeding since childhood and menorrhagia and bleeding per vaginum after puberty, also had history of excessive blood loss after birth of child. Her coagulation profile was normal but platelet function testing by platelet aggregation assay showed abnormal aggregation of platelet with epinephrine. This type of response is seen in "Quebec platelet disorder" which is a rare autosomal dominant disorder of platelet function characterized by increased bleeding after any injury or trauma.

摘要

在此报告一例魁北克血小板疾病。一名33岁女性自幼有鼻出血和牙龈出血史,青春期后出现月经过多和阴道出血,产后也有失血过多史。她的凝血指标正常,但通过血小板聚集试验进行的血小板功能检测显示,血小板对肾上腺素的聚集异常。这种反应见于“魁北克血小板疾病”,这是一种罕见的常染色体显性血小板功能障碍疾病,其特征是任何损伤或创伤后出血增加。

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