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血栓性血小板减少性紫癜患者血浆置换前后ADAMTS13抗原变化及临床意义 被引量：5

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摘要 血栓性血小板减少性紫癜（TTP）是一种罕见的微血管血栓——出血综合征。该病进展迅速，临床表现多样，病死率高。TTP典型临床表现为Moschcowitz’s五联征：血小板减少、溶血性贫血、发热、中枢神经系统和肾脏受累表现。现已证实血管性血友病因子裂解蛋白酶（ADAMTS13）缺陷，导致超大分子量vWF多聚体（ULvWF）不能被裂解而引起血小板聚集、微血管血栓形成是TTP的重要发病机制。血浆置换（PE）是治疗该病的一种安全有效的方法，使患者的病死率由90％以上降至25％。
作者 杨鹏 李燕萍 闻慧琴 张循善 夏雪
机构地区 安徽医科大学第一附属医院输血科
出处 《中华血液学杂志》 CAS CSCD 北大核心 2012年第10期879-880,共2页 Chinese Journal of Hematology
关键词 血栓性血小板减少性紫癜 ADAMTS13 血浆置换 血管性血友病因子裂解蛋白酶 临床意义 患者 微血管血栓形成 抗原
分类号 R554.6 [医药卫生—血液循环系统疾病]
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参考文献9

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二级参考文献31

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共引文献11

同被引文献15

引证文献5

二级引证文献20

中华血液学杂志
2012年 第10期

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