阵发性睡眠性血红蛋白尿病人骨髓红系和粒－单系祖细胞的特点

本文采用造血祖细胞体外培养技术观察了阵发性睡眠性血红蛋白尿（ＰＮＨ）病人骨髓红系祖细胞（ＢＦＵ一Ｅ和ＣＦＵ一Ｅ）和粒一单系祖细胞（ＣＦＵ一ＧＭ）的增殖能力；骨髓细胞经酸化ＡＢ型血清处理后的ＢＦＵ一Ｅ，ＣＦＵ一Ｅ和ＣＦＵ一ＧＭ的增殖能力；以及ＢＦＵ一Ｅ、ＣＦＵ一Ｅ对红细胞生成素（Ｅｐｏ）和（ＣＦＵ一ＧＭ对粒一单系集落刺激因子（ＧＭ一ＣＳＦ）的反应能力，发现ＰＮＨ病人骨髓ＢＦＵ一Ｅ，ＣＦＵ一Ｅ和ＣＦＵ一ＧＭ集落数明显低于正常；骨髓细胞经新鲜酸化ＡＢ型血清处理后培养的ＢＦＵ一Ｅ、ＣＦＵ一Ｅ和ＣＦＵ一ＧＭ集落数明显低于经热灭活酸化ＡＢ型血清处理后培养的集落数；以及ＢＦＵ一Ｅ，ＣＦＵ一Ｅ对Ｅｐｏ和ＣＦＵ一ＧＭ对ＧＭ一ＣＳＦ的剂量反应曲线低平。因此认为ＰＮＨ病人骨髓红系和粒一单系祖细胞有以下特点：１．增殖能力降低：２．在酸性条件下对补体的敏感性增加；３．对造血因子的敏感性降低。

Characteristics of hematopoietic progenitors of marrow cells in patients with paroxysmal nocturnal hemoglobinuria

The hematopoietic progenitor culture method was used to investigate theproliferative capacity of erythroid progenitors(BFU-E and CFU-E)andgranulocyte-macrophage progenitors(CFU-GM) and that of BFU-E, CFU-E andCFU-GM in marrow cells exposed to acidified fresh human AB serum, and the responsesof BFU-E,CFU-E to erythropoietin(Epo) and CFU-GM to granulocyte-macrophage col-ony stimulating factor(GM-CSF) in patients with PNH, It was found that the number ofBFU-E,CFu-E and CFU-GM of marrow cells ih patients with PNH was much moredecrease than that of the normal.The number of BFU-E.CFU-E.and CFU-GM of mar-row cells exposed to acidiffed fresh human AB serum was much lower than that exposed toacidified heat inactivated human AB serum in PNH. The dose response curve of theBFU-E,CFU-Ecolony to Epo and CFU-GM to GM-CSF in PNH was lower and moreeven than that of the normal.These results suggested that in patients with PNH BFU-E,CFU-E and CFU-GM showed decreased proliferative capacity,increased sensitivity tocomplement and declined response to hematopoietic growth factor.