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采用造血祖细胞体外培养技术研究了阵发性睡眠性血红蛋白尿症（ＰＮＨ）患者骨髓红系祖细胞（ＢＦＵ－Ｅ和ＣＦＵ－Ｅ）的增殖能力；骨髓细胞经新鲜酸化ＡＢ型血清处理后培养的ＢＦＵ－Ｅ和ＣＦＵ－Ｅ的增殖能力；以及ＢＦＵ－Ｅ和ＣＦＵ－Ｅ对红细胞生成素（Ｅｐｏ）的反应能力。发现ＰＮＨ患者骨髓ＢＦＵ－Ｅ和ＣＦＵ－Ｅ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－Ｅ和ＣＦＵ－Ｅ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；ＢＦＵ－Ｅ和ＣＦＵ－Ｅ对Ｅｐｏ的反应回归直线呈低平状态。认为ＰＮＨ患者骨髓红系祖细胞膜缺陷可导致其在酸性条件下对补体的敏感性增高和导致其对Ｅｐｏ的敏感性降低。
The proliferative ability of bone marrow erythroid progenitor cells (BFU-E and CFU-E) in patients with paroxysmal nocturnal hemoglobinuria (PNH) was studied using in vitro culture of hematopoietic progenitor cells. Bone marrow cells were cultured with fresh acidified AB serum The proliferative capacity of BFU-E and CFU-E; and the ability of BFU-E and CFU-E to respond to erythropoietin (Epo). The number of BFU-E and CFU-E colonies in the bone marrow of patients with PNH was significantly lower than that of the normal group. The numbers of BFU-E and CFU-E colonies of bone marrow cells cultured with fresh acidified AB serum were significantly lower than those of heat-inactivated AB serum After treatment, the number of colonies cultured; the reaction of BFU-E and CFU-E to Epo regression line was low flat state. It is believed that membrane defects of myeloid erythroid progenitor cells in PNH patients lead to their increased sensitivity to complement under acidic conditions and to their reduced susceptibility to Epo.