Terasawa T, Shichishima T, Matsuda M, Uchida T, Kariyone S
Department of Physiology, Ohu University, School of Dentistry, Fukushimaken, Japan.
Br J Haematol. 1990 Jul;75(3):428-32. doi: 10.1111/j.1365-2141.1990.tb04359.x.
In order to investigate erythropoiesis in paroxysmal nocturnal haemoglobinuria (PNH), the relative proportions of complement-mediated lysed erythroblasts in individual bursts of two cases of PNH were analysed by a modified complement lysis sensitivity test using the trypan blue dye exclusion method. All bursts contained both complement-sensitive and complement-insensitive erythroblasts and the ratio between them varied from burst to burst. The means +/- SD of the haemolytic percentages of individual bursts in cases 1 and 2 were 30.9 +/- 10.8% (n = 20, ranging from 13.2% to 52.8%) and 54.8 +/- 16.4% (n = 31, ranging from 26.4% to 82.5%), respectively. In addition, the cumulative frequency distributions of the proportions of complement-mediated lysed erythroblasts in individual bursts approached normal frequency distributions. These results suggest that two red cell populations with different susceptibilities to complement in PNH are derived from a single population of erythropoietic progenitor cells rather than a dual population of normal and PNH erythropoietic progenitor cells.
为了研究阵发性夜间血红蛋白尿（PNH）中的红细胞生成情况，采用台盼蓝染料排除法，通过改良的补体溶解敏感性试验分析了2例PNH患者单个红系爆式集落中补体介导溶解的成红细胞的相对比例。所有爆式集落均同时含有补体敏感和成红细胞及补体不敏感的成红细胞，且它们之间的比例因爆式集落而异。病例1和病例2中单个爆式集落溶血百分比的均值±标准差分别为30.9±10.8%（n = 20，范围为13.2%至52.8%）和54.8±16.4%（n = 31，范围为26.4%至82.5%）。此外，单个爆式集落中补体介导溶解的成红细胞比例的累积频率分布接近正态频率分布。这些结果表明，PNH中对补体敏感性不同的两个红细胞群体源自单个红系祖细胞群体，而非正常和PNH红系祖细胞的双群体。
