Raskind W H, Ferraris A M, Najfeld V, Jacobson R J, Moohr J W, Fialkow P J
Department of Medicine, University of Washington School of Medicine, Seattle 98195.
Leukemia. 1993 Aug;7(8):1163-7.
The Ph chromosome abnormality is involved in the pathogenesis of almost all patients with chronic myelocytic leukemia (CML). Previous studies on the B-lymphoid cell lineage in two patients with Ph-positive CML suggest that there may also be a clonal Ph-negative stage in CML and that the Ph-positive stage arises by subclonal expansion. To determine whether this is a frequent or a rare occurrence, 14 additional glucose-6-phosphate dehydrogenase (G6PD)-heterozygous patients with CML were studied. In five of these patients there was a statistically significant excess of Ph-negative B-lymphoid cell lines expressing the same G6PD type expressed in the corresponding CML clone. In no case was an excess of B-lymphoid lines expressing the opposite G6PD type recovered. These data provide further evidence that in some patients the Ph chromosome arises in a pluripotent stem cell from a pre-existing Ph-negative clone that enjoys a growth advantage.
Ph染色体异常几乎涉及所有慢性粒细胞白血病（CML）患者的发病机制。先前对两名Ph阳性CML患者的B淋巴细胞谱系研究表明，CML中可能还存在一个克隆性Ph阴性阶段，且Ph阳性阶段是通过亚克隆扩增产生的。为了确定这种情况是常见还是罕见，我们又研究了另外14名葡萄糖-6-磷酸脱氢酶（G6PD）杂合的CML患者。在其中5名患者中，表达与相应CML克隆中相同G6PD类型的Ph阴性B淋巴细胞系在统计学上显著过量。在任何情况下，均未回收表达相反G6PD类型的过量B淋巴细胞系。这些数据进一步证明，在一些患者中，Ph染色体起源于一个具有生长优势的预先存在的Ph阴性克隆的多能干细胞。
