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浅谈对1例阵发性睡眠性血红蛋白尿患者进行护理干预的方法

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摘要 阵发性睡眠性血红蛋白尿(paroxysmal noctumal helnoglobinuria,PNH)是一种获得性造血干细胞克隆缺陷性疾病。该病的发病机理是患者病变细胞的X染色体上PIG-A基因发生突变,引起其GPI蛋白(GPI-AP)表达减少或缺失,从而导致其血细胞(红细胞粒细胞及血小板)膜对补体异常敏感而被破坏,进而使患者出现持续性血管内溶血。该病患者的临床表现是可出现与睡眠有关的间歇发作的血红蛋白尿、溶血性贫血、血栓和造血功能障碍等[1]。
出处 《当代医药论丛》 2015年第14期99-100,共2页
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