阵发性睡眠性血红蛋白尿患者骨髓粒单系祖细胞的体外培养研究

In Vitro Culture of Granulocyte-Macrophage Progenitors in Marrow Cells from Patients with Paroxysmal Nocturnal Hemoglobinuria

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 vitro hematopoietic progenitor culture method was used to study the proliferative capacity of grnulocyte-macrophage progenitors(colony forming units-granulocyte-macrophage or CFU-GM) in marrow cells from patients with paroxysmal nocturnal hemoglobinuria(PNH); the proliferative capacity of CFU-GM in marrow cells exposed to acidified fresh human AB serum; and the response of CFU-GM in marrow cells from patients with PNH to granulocyte-macrophage colony stimulating factor(GM-CSF). We found that the number of CFU-GM in marrow cells from patients with PNH was greater decrease than that from normal;that the number of CFU-GM in marrow cells exposed to acidified fresh human AB serum was significantly lower than that exposed to acidified heat-inactivated human AB serum; and that the dose response curve of the colony of CFU-GM in marrow cells from patients with PNH to GM-CSF was lower and more even than that from normal. These results suggest that in patients with PNH whose CFU-GM expres sed membrane abnormality similar to that of erythrocytes. The abnormality results in the complement-mediated injury or lysis to CFU-GM on the condition of acdified fresh human AB serum and in sensitivity decvease to GM-CSF.