Sadamori N, Gomez G A, Sandberg A A
Blood. 1983 May;61(5):935-9.
To assess parameters of therapeutic response and of survival after the onset of the blastic phase (BP) in 64 patients with Ph1-positive chronic myeloid leukemia (CML), a number of clinical, hematologic, and cytogenetic data at the BP were evaluated. Among 10 parameters checked, only the chromosomal findings correlated with the therapeutic response and survival after the onset of the BP. The patients were divided into three groups on the basis of the chromosome findings in the bone marrow, blood, and spleen: (1) those with only a Ph1 (PP), (2) those having two types of clones, i.e., one clone with only a Ph1 and another with additional karyotypic changes (AP), and (3) those with only abnormal clones in addition to the Ph1 (AA). The number of patients in each group was 29 in PP, 15 in AP, and 20 in AA. The results were as follows. (1) The percentage of patients with a good therapeutic response was 79% (23/29) in PP, 53% (8/15) in AP, and 30% (6/20) in AA. (2) The median survival after the onset of the BP was 171 days (5.7 mo) in PP, 146 days (4.9 mo) in AP, and 74 days (2.5 mo) in AA. Statistically, there was a significant difference between the AA and the other two groups (p less than 0.05). For further study, the AA and AP patients were divided into 4 subgroups each: those with 48 or more chromosomes, those with 47 chromosomes, those with pseudodiploidy, and those with hypodiploidy. A subgroup with 48 or more chromosomes in the AA patients had a very short survival (median, 25 days; 0.8 mo) and a poor therapeutic response (1/9, 11%). Our observations suggest that the lack of a clone with only a Ph1 (AA), particularly with more than 48 chromosomes, at the acute crisis or shortly after the onset of the BP indicates an unfavorable therapeutic response and a poor prognosis after the onset of the BP.
为评估64例Ph1阳性慢性粒细胞白血病（CML）患者进入急变期（BP）后的治疗反应参数及生存情况，我们对BP期的多项临床、血液学和细胞遗传学数据进行了评估。在检查的10项参数中，只有染色体检查结果与BP期的治疗反应及生存情况相关。根据骨髓、血液和脾脏的染色体检查结果，将患者分为三组：（1）仅含Ph1的患者（PP）；（2）有两种克隆类型的患者，即一种仅含Ph1的克隆，另一种伴有额外核型改变的克隆（AP）；（3）除Ph1外仅含异常克隆的患者（AA）。每组患者人数分别为：PP组29例，AP组15例，AA组20例。结果如下：（1）PP组治疗反应良好的患者比例为79%（23/29），AP组为53%（8/15），AA组为30%（6/20）。（2）BP期开始后的中位生存期，PP组为171天（5.7个月），AP组为146天（4.9个月），AA组为74天（2.5个月）。统计学分析显示，AA组与其他两组之间存在显著差异（p<0.05）。为进一步研究，将AA组和AP组患者各分为4个亚组：染色体数为48条或更多的患者、染色体数为47条的患者、假二倍体患者和亚二倍体患者。AA组中染色体数为48条或更多的亚组生存期很短（中位生存期25天；0.8个月），治疗反应差（1/9，11%）。我们的观察结果表明，在急性危象期或BP期开始后不久，缺乏仅含Ph1的克隆（AA），尤其是染色体数超过48条时，提示BP期开始后的治疗反应不佳且预后不良。
