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本文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(PNH)病人骨髓粒单系祖细胞(CFU-GM)的增殖能力;骨髓细胞经酸化AB型血清处理后CFU-GM的增殖能力;和CFU-GM对粒单系集落刺激因子(GM-CSF)的反应能力,发现PNH病人骨髓CFU-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CFU-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;CFU-GM对GM-CSF的剂量反应曲线低平。因此认为PNH病人骨髓粒单系祖细胞膜缺陷导致其在酸性条件下对补体的敏感性增高而致损伤或溶解和导致其对粒单系集落刺激因子的敏感性降低。
In this study, the proliferation of myelomonocytic myelomonocytic progenitor cells (CFU-GM) in patients with paroxysmal nocturnal hemoglobinuria (CFH-GM) was studied using in vitro culture of hematopoietic progenitor cells. The proliferation of CFU-GM (CFU-GM) on the response to GM-CSF. The number of CFU-GM colonies in the bone marrow of patients with PNH was significantly lower than that of the normal controls. The bone marrow cells were treated with fresh acidified AB serum The number of GM-CSF colonies was significantly lower than that of heat-inactivated AB-type serum. The dose response curve of CFU-GM to GM-CSF was low. Therefore, it is thought that membrane defects of myelomonocytic progenitor cells in PNH patients result in increased susceptibility to complement in acidic conditions to cause damage or lysis and lead to reduced sensitivity to granulocyte colony stimulating factor.