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[阵发性睡眠性血红蛋白尿症及再生障碍性贫血-阵发性睡眠性血红蛋白尿症综合征中补体敏感红细胞的评估与定量分析]

[Evaluation and quantification of complement-sensitive red cells in PNH and aplastic anemia-PNH syndrome].

作者信息

Shichishima T, Terasawa T, Uchida T, Kariyone S

出版信息

Rinsho Ketsueki. 1989 Mar;30(3):297-302.

PMID:2769953
Abstract

In order to clarify the erythropoiesis of complement-sensitive red cells in paroxysmal nocturnal hemoglobinuria (PNH), the proportion of complement-sensitive red cells and compositional classification of examined red cells were investigated by means of complement lysis sensitivity test in 27 patients with initial diagnosis of PNH and 17 patients with aplastic anemia-PNH syndrome, and their bone marrow nucleated cell counts were also compared. The proportion of complement-sensitive erythrocytes was 41.0 +/- 22.0% (n = 26) in PNH and 29. 5 +/- 15.0% (n = 17) in aplastic anemia-PNH syndrome, and no significant difference was recognized between them. The nucleated cell count at the time of PNH diagnosis was 19.1 +/- 12.5 X 10(4)/microliters (n = 21) in PNH and 12.6 +/- 8.8 X 10(4)/microliters (n = 12) in aplastic anemia-PNH syndrome, and no significant difference between them was apparent. These findings suggest that the erythropoiesis of complement-sensitive red cells shows a similarity between PNH at initial occurrence and aplastic anemia-PNH syndrome, once PNH has occurred.

摘要

为了阐明阵发性夜间血红蛋白尿(PNH)中补体敏感红细胞的红细胞生成情况,通过补体溶解敏感性试验,对27例初诊PNH患者和17例再生障碍性贫血-PNH综合征患者的补体敏感红细胞比例及所检测红细胞的组成分类进行了研究,并比较了他们的骨髓有核细胞计数。PNH患者补体敏感红细胞比例为41.0±22.0%(n = 26),再生障碍性贫血-PNH综合征患者为29.5±15.0%(n = 17),两者之间无显著差异。PNH诊断时的有核细胞计数,PNH患者为19.1±12.5×10⁴/微升(n = 21),再生障碍性贫血-PNH综合征患者为12.6±8.8×10⁴/微升(n = 12),两者之间无明显差异。这些发现表明,一旦PNH发生,补体敏感红细胞的红细胞生成在初发PNH和再生障碍性贫血-PNH综合征之间表现出相似性。

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