PNH患者骨髓红系祖细胞体外培养的研究

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。

IN VITRO CULTURE OF ERYTHROID PROGENITOR IN MARROW CELLS FROM PATIENTS WITHPAROXYSMAL NOCTURNAL HEMOGLOBINURIA

n vitro hematopoietic progenitor culture was used tostudy the proliferative capacity of erythroid progeni-tor ,burst forming units-erythroid (BFUE)and colonyforming units erythroid(CFU-E ) in marrow cells frompatients with paroxysmal nocturnal hemoglobinuria(PNH) , the proliferative capacity of BFUE and CFU-E in marrow cells exposed to acidified fresh human ABserum , and the response of BFU-E and CFU-E inPNH marrow cells to erythropoietin(Epo). It wasfound that BFU-E and CFU-E in PNH marrow cells was much lower than that from normal ,the BFU-Eand CFU-E in marrow cells exposed to acidified fresh human AB serum were significantly lower than that ex-posed to heat-inactivated acidified human AB serum,the dose response curve of BFU-E and CFU-E in PNHmarrow cells to Epo was lower and more even thanthat from normal.These results suggested that BFU-Eand CFU-E in patients with PNH expressed the mem-brane abnormality similar to that on erythrocytes.This abnormality resulted in the high complement-sen-sitivity of BFU-E and CFU-E to acidified fresh humanAB serutn and low sensitivity to Epo.