• 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Platelets >Absence of bleeding upon dual antiplatelet therapy in a patient with a immune GPVI deficiency
【24h】

Absence of bleeding upon dual antiplatelet therapy in a patient with a immune GPVI deficiency

机译：患有免疫GPVI缺乏的患者的双抗血小板治疗缺乏出血

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Acquired deficiencies in platelet glycoprotein VI are rare and have not been found associated with other defects. Here we report the case of a 64-year old male patient presenting an immune GPVI deficiency associated to a mutation in the alpha-actinin gene and who has been treated with dual anti platelet therapy without bleeding. Introduction: Glycoprotein (GP) VI, a pluripotent receptor interacting with collagen and fibrin(ogen) is responsible for thrombus formation, growth and stability (1-4). It is co-expressed with the Fc receptor gamma (FcR gamma) chain (5). GPVI is not critical for haemostasis since subjects with a GPVI deficiency usually present low or even no bleeding tendency (6, 7). Acquired GPVI deficiency due to antibody-induced GPVI depletion is the most frequent finding. At least 10 patients have been described with an acquired GPVI deficiency, most often associated to immune thrombocytopenia, moderate bleeding and impaired collagen-induced platelet aggregation (7). Several mechanisms leading to the GPVI deficiency are proposed including antibody-triggered GPVI internalization and/or shedding of the extracellular domain (8, 9).We report the case of a patient presenting an acquired GPVI deficiency different from those previously described: (i) he is male whereas all previous cases were female, (ii) he is heterozygous for a mutation in alpha (alpha)-actinin-1 gene and (iii) he was treated with dual antiplatelet therapy with no haemorrhagic manifestation.
机译：获得性血小板糖蛋白VI缺陷是罕见的，尚未发现与其他缺陷相关。我们在此报告一例64岁男性患者，其表现为与α-肌动蛋白基因突变相关的免疫GPVI缺乏症，并接受了双重抗血小板治疗，无出血。简介：糖蛋白（GP）VI是一种与胶原和纤维蛋白（原）相互作用的多功能受体，负责血栓的形成、生长和稳定性（1-4）。它与Fc受体γ（FcRγ）链（5）共表达。GPVI对止血并不重要，因为GPVI缺乏的受试者通常表现出较低甚至没有出血倾向（6,7）。最常见的发现是由于抗体诱导的GPVI缺失导致的获得性GPVI缺乏。至少有10名患者被描述为获得性GPVI缺乏症，通常与免疫性血小板减少症、中度出血和胶原诱导的血小板聚集受损有关（7）。提出了导致GPVI缺乏的几种机制，包括抗体触发的GPVI内化和/或胞外结构域脱落（8,9）。我们报告了一例患者出现与之前描述的不同的获得性GPVI缺乏症：（i）他是男性，而之前的所有病例都是女性，（ii）他是α（α）-肌动蛋白-1基因突变的杂合子，（iii）他接受了双重抗血小板治疗，没有出血表现。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号