Paroxysmal nocturnal hemoglobinuria. A complement-mediated disease.
Paroxysmal nocturnal hemoglobinuria. A complement-mediated disease.
复制标题
阵发性睡眠性血红蛋白尿。
DOI：
10.1159/000463072
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发表时间：
1989
期刊：
影响因子：
--
通讯作者：
Nicholson-Weller,A
中科院分区：
文献类型：
--
作者：
Halperin,JA;Nicholson-Weller,A
阵发性睡眠性血红蛋白尿(PNH)是一种以红细胞对补体溶血作用敏感性增加为特征的溶血性疾病。PNH患者异常血细胞缺乏两种保护正常红细胞免受补体溶血作用的膜蛋白，即衰变加速因子和C8结合蛋白。其他与补体调节无关的膜蛋白，与衰变加速因子和C8结合蛋白具有共同的翻译后修饰，即与细胞膜的糖磷脂酰肌醇连接，也在PNH细胞中缺失。本文对PNH的临床、生物学和分子方面进行了讨论。此外，还讨论了临床应用中的诊断试验，并提出了使用间接免疫荧光分析的新试验。
Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic disease characterized by an increased sensitivity of erythrocytes to the hemolytic action of complement. Two membrane proteins, the decay-accelerating factor and the C8-binding protein, which protect normal erythrocytes from the hemolytic action of complement, are deficient on the abnormal blood cells from patients with PNH. Other membrane proteins unrelated to complement regulation, but which share with the decay-accelerating factor and the C8-binding protein a common post-translational modification, namely a glycan-phosphatidylinositol linkage to the cell membrane, are also missing from PNH cells. In the present review, clinical, biological, and molecular aspects of PNH are discussed. In addition, diagnostic tests in clinical use are discussed, and new tests using indirect immunofluorescent assays are proposed.