初治青少年及年轻成人急性淋巴细胞白血病的临床特征及预后研究

目的探讨初治青少年及年轻成人(AYAs)急性淋巴细胞白血病(急淋)的临床及MICM分型特征,及其对生存的影响。方法回顾性研究2000年1月—2011年6月收治的100例11～45岁初治AYAs急淋患者的MICM分型、临床特征及疗效,应用SPSS 17.0软件进行统计学分析。结果 100例AYAs急淋患者中,11～30岁者48例,31～45岁者52例,两组临床及MICM分型特征比较差异均无统计学意义(P>0.05);初诊时外周血白细胞计数(WBC)≥50×109/L者38例,WBC与预后有关(P=0.006);初诊时中枢神经系统白血病(CNSL)及睾丸白血病(TL)共2例,影响长期生存(P=0.000);普通B细胞急淋(common-B-ALL)58例、前B细胞急淋(pre-B-ALL)12例、早前B细胞急淋(pro-B-ALL)5例、成熟B细胞急淋(成熟B-ALL)1例、T细胞急淋(T-ALL)23例、伴髓系抗原表达的急淋(My+-ALL)3例,CD10(+)ALL、CD10(-)ALL、T-ALL、My+-ALL组间长期生存率比较差异有统计学意义(P=0.025);细胞遗传学检查显示亚(假)二倍体6例、t(9;22)/bcr-abl 3例、t(4;11)/MLL-AF4 1例、超二倍体8例、t(12;21)/TEL-AML1 1例。亚(假)二倍体、t(9;22)/bcr-abl、t(4;11)/MLL-AF4预后较差(P=0.000);泼尼松预试验反应不良者11例、初治诱导化疗效果未达到完全缓解(CR)者9例,早期治疗反应与预后相关(P<0.05)。结论初诊时外周血WBC及CNSL/TL、泼尼松预试验反应、初治诱导化疗效果、免疫分型及细胞遗传学特征对AYAs急淋预后评估具有重要意义;不同年龄的AYAs急淋具有相同的临床及MICM分型特征,年龄不是AYAs急淋的预后影响因素。

Clinical Characteristics and Prognostic Analysis of Adolescents and Young Adults with Newly Diagnosed Acute Lymphoblastic Leukemia

Objective To investigate the clinical characteristics,MICM classification and prognostic factors of adolescents and young adults with newly diagnosed acute lymphoblastic leukemia.Methods The clinical characteristics,MICM classification and efficacy of 100 AYAs aged from 11 to 45 years with newly diagnosed acute lymphoblastic leukemia,who admitted during the period from January 2000 to June 2011,were analyzed by SPSS 17.0.Results In the 100 AYAs,forty-eight patients were aged 11~30 years,fifty-two were aged 31~45 years,the clinical characteristics and MICM classification showed no statistically significant differences between the two groups(P>0.05).Patients with high leukocyte counts(≥50×109/L)at diagnosis accounted for 38%,WBC count was related to prognosis(P=0.006).Central nervous system leukemia and/or testicles leukemia(CNSL/TL)at diagnosis accounted for 2%,CNSL/TL affected overall survival(OS)(P=0.000).Among the 100 patients,58 were Common-B-ALL,12 were pre-B-ALL,5 were pro-B-ALL,1 was mature B-ALL,23 were T-ALL and 3 were My+-ALL,and there was statistically significant differences in immunophnotypes(CD10 positive ALL,CD10 negative ALL,T-ALL,My+-ALL),P=0.025.Cytogenetics features were associated with overall survival(OS)(P=0.000),poor-risk cytogenetics included hypodiploidy or pseudodiploid(n=6),t(9;22)/bcr-abl(n=3),and t(4;11)/MLL-AF4(n=1).Standard-risk cytogenetics included normal karyotype,hyper diploid(n=8),t(12;21)/TEL-AML(n=1).11 cases had poor response to prednisone prophase and 9 cases had slow response to inducing chemotherapy.Poor early response to therapy was related to prognosis(P<0.05).Conclusion High WBC count and CNSL/TL at diagnosis,cyto genetic features,immunophnotype,and slow response to prednisone or chemotherapy have important prognostic significance to AYAs with acute lymphoblastic leukemia.The clinical and biological features are similar in patients with different ages.Age is not a significant prognostic factor in this group of AYAs.