Eaves A C, Eaves C J
Exp Hematol. 1979;7 Suppl 5:65-75.
Peripheral blood and bone marrow specimens from 6 patients with Ph1-positive CML were evaluated for their content of erythropoietic and granulopoietic colony-forming progenitor cells. Specimens obtained from untreated patients showed marked increases in all compartments the most dramatic of which were for CFU-E and circulating CFU-C. This increased cell flow down the early stages of the red cell pathway in CML suggests that heightened proliferation and differentiation of primitive hemopoietic cells may be a more general phenomenon than previously suspected in this disease. In 5 of 6 patients, abnormal erythroid progenitors capable of proliferation and differentiation into hemoglobinized erythroblasts in cultures containing less than 0.002 units of erythropoietin/ml were regularly detected. In the 6th patient abnormal growth was not seen in cultures of the initial marrow obtained but was detected in cultures set up with peripheral blood taken 7 months later. The unexplained amplification of the erythropoietic compartment and the ability of some of these cells to mature in vitro in the virtual absence of erythropoietin is at variance with the anemia characteristic of untreated patients. This suggests the possibility of a major differentiation block at the level of CFU-E. Further studies of the properties of erythroid progenitors in these patients should help to provide new insights into the pathogenesis of CML and may provide useful markers for monitoring engrafted cell function after autotransplantation of patients in blast crisis.
对6例Ph1阳性慢性粒细胞白血病患者的外周血和骨髓标本进行了评估,以检测其中红细胞生成和粒细胞生成集落形成祖细胞的含量。从未经治疗的患者获取的标本显示所有细胞成分均显著增加,其中最明显的是红细胞集落形成单位(CFU-E)和循环中的粒细胞集落形成单位(CFU-C)。慢性粒细胞白血病患者红细胞生成途径早期阶段细胞流量增加,这表明原始造血细胞增殖和分化增强可能是该疾病中比先前怀疑的更为普遍的现象。在6例患者中的5例中,在每毫升含少于0.002单位促红细胞生成素的培养物中,能够增殖并分化为血红蛋白化红细胞的异常红系祖细胞被定期检测到。在第6例患者中,最初获取的骨髓培养物中未见异常生长,但在7个月后采集的外周血建立的培养物中检测到了异常生长。促红细胞生成成分的不明扩增以及其中一些细胞在几乎没有促红细胞生成素的情况下在体外成熟的能力与未经治疗患者的贫血特征不符。这提示在CFU-E水平可能存在主要的分化阻滞。对这些患者红系祖细胞特性的进一步研究应有助于为慢性粒细胞白血病的发病机制提供新的见解,并可能为监测急变期患者自体移植后植入细胞的功能提供有用的标志物。