探讨急性淋巴细胞白血病患者费城染色体阳性的生物学特征及临床预后

Biological features and clinical prognosis in patients with acute lymphoblastic leukemia and philadelphia chromosomepositive

目的分析费城(Ph)染色体阳性(+)急性淋巴细胞白血病(ALL)的发生率,探讨其生物学特征与临床预后的关系。方法应用G显带的常规细胞遗传学技术对300例ALL患者进行检测,其中Ph染色体(+)患者36例,Ph染色体阴性(-)患者264例,分析Ph染色体(+)组患者与Ph染色体(-)组患者的生物学特征、缓解率、生存期差异。结果 300例ALL初诊患者发现Ph染色体(+)患者36例,Ph染色体阳性率为12.0%。Ph(+)组患者的中位血红蛋白、血小板计数、外周血和骨髓幼稚淋巴细胞比例与Ph染色体(-)组患者比较,差异均无统计学意义(P>0.05);但Ph(+)组患者的白细胞计数明显高于Ph(-)组患者,差异有统计学意义(P<0.05)。36例Ph染色体(+)患者免疫分型资料显示:前B-ALL 9例、My+-ALL 5例、Common-ALL 22例。预后分析显示:Ph(+)组ALL患者和Ph(-)组ALL患者的第1次缓解率分别为52.8%(19/36)和84.8%(224/264),两组比较,差异有统计学意义(χ2=29.89,P<0.05);两组复发率分别为57.9%(11/19)和25.4%(57/224),两组比较,差异有统计学意义(χ2=22.13,P<0.05)。两组患者随访期内中位生存期分别为8、34个月。结论 Ph染色体(+)ALL患者比Ph染色体(-)ALL患者预后更差,生存期短,临床上要注意根据Ph染色体阳性与否采取相应的治疗策略。

Objective To analyze the incidence of Philadelphia chromosome（Ph）-positive in the patients with acute lymphoblastic leukemia（ALL）,and to discuss the relationship between the biological features and clinical prognosis.Methods The G banding conventional cytogenetic technology was used to test 300 ALL patients,including 36 patients with the Ph chromosome（＋）,264 patients with Ph chromosome（-）.The differences in the biology characteristic,remission rate and survival period between the Ph（＋）group and the Ph（-）group were analyzed.Results Among 300 cases of newly diagnosed ALL,36 cases were found Ph chromosome（＋）with the Ph chromosome positive rate of 12.0%.The median of hemoglobin,platelet count and percentage of immature lymphocyte in peripheral blood and bone marrow had no statistically significant differences between the Ph（＋）group and Ph（-）group（P〉0.05）;but white blood cell（WBC）count was obviously higher than that of Ph（-）group,the difference was statistically significant（P〈0.05）.The immune classification data in 36 cases of Ph（＋）showed that pro-B-ALL was in 9cases,My＋-ALL was in 5cases and common-ALL was in 22 cases.The prognostic analysis showed that first time remission rate in the Ph（＋）and Ph（-）groups were 52.8%（19/36）and 84.8%（224/264）respectively,the difference between the two groups was statistically significant（χ^2= 29.89,P〈0.05）;the recurrence rates in the two groups were 57.9%（11/19）and 25.4%（57/224）respectively,the difference between the two groups was statistically significant（χ^2=22.13,P〈0.05）.The median lifetime of the Ph（＋）and Ph（-）groups were 8months and 34 months respectively.Conclusion The ALL patients with Ph（＋）have poorer prognosis and shorter survival period than those with Ph（-）,therefore clinic should adopt the corresponding treatment strategies according to whether Ph positive or not.