EVI1基因阳性的儿童急性髓系白血病临床分析  

Clinical analysis of pediatric acute myeloid leukemia with EVI1 gene positive

作　　者：邱奕宁[1] 郝琏琏 余慧[1] 周芬[1] 蔡馥丞[1] 徐佳伟[1] 金润铭[1] 

机构地区：[1]华中科技大学同济医学院附属协和医院儿科,武汉430022

出　　处：《中华实用儿科临床杂志》2013年第21期1656-1659,共4页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的分析伴有EVI1基因表达的儿童急性髓系白血病（AML）的临床特点、疗效及预后。方法采用多重巢式反转录聚合酶链反应（RT—PCR）对2009年1月至2011年12月收治的AML患儿进行融合基因检测，确定EVI1的表达情况，并追踪其临床特征及疗效，并与EVI1（-）组AML患儿进行对比分析。结果EVI1基因在83例AML患儿中的表达率为15．65％（13／83例），在高危患儿中表达率最高，且易伴有复杂核型、MLL重排、-7等预后不良的细胞分子遗传学改变。EVI1（＋）组诱导缓解率为45．5％，明显低于EVI1（-）组（79．3％）（／=5．497，P〈0．05），尤其1个疗程达到缓解者比率显著低于EVI1（-）组（18．2％比63．8％,X2=7．828，P〈0．01）。虽然2组病死率无显著差异，但是EVI1（＋）组早期病死率显著高于EVI1（-）组（45．5％比8．6％，P〈0．01）。EVI1（＋）组4年无事件生存率（EFS）显著低于EVI1（-）组[（21．2±13．8）％比（60．2±9．1）％,X2=4．493，P〈0．05]。而EVI1（＋）组4年累积总生存率亦显著低于EVI1（-）组[（32．4±7．1）％比（63．3±10．9）％,X2=4．602，P〈0．05]。通过多因素及单因素二分类Logistic回归分析显示，EVI1阳性与否与预后均无显著相关性。结论EVI1基因阳性表达的AML患儿病情进展快，缓解率低，早期病死率高，EFS低，故EVI1基因表达是儿童AML预后不良的一项指标，但尚不能作为独立预后不良因子。目的分析伴有EVIl基因表达的儿童急性髓系白血病（AML）的临床特点、疗效及预后。方法采用多重巢式反转录聚合酶链反应（RT—PCR）对2009年1月至2011年12月收治的AML患儿进行融合基因检测，确定EVIl的表达情况，并追踪其临床特征及疗效，并与EVIl（-）组AML患儿进行对比分析。结果EVIl基因在83例AML患儿中的表达率为15．65％（13／83例），在高危患儿中表达率最高，且�Objective To analyze the clinical features and prognosis of pediatric acute myeloid leukemia （AML） with EVIl gene positive. Methods The nested RT-PCR was performed to detect the EVIl expression in pe- diatric AML patients from Jan. 2009 to Dec. 2011. The patients with EVI! were investigated on clinical features, curative effects and prognosis. The differences between EVIl （ ＋ ） and EVIl （ - ） patients were also analyzed. Results The fre- quency of EVIl （ ＋ ） expression was 15.65% （13/83 cases）in pediatric AML,with the highest incidence in high-risk patients. EVIl （ ＋ ） was obviously associated with some unfavorable molecular genetic changes such as complex karyo- type, MLL rearrangement, and monosomy 7. There were significant differences for EVIl （ ＋ ） group and EVIl （ - ） group in the complete remission rate （45.5% vs 79.3% ,X2 = 5. 497 ,P 〈 0.05） and complete remission（CR） rate af- ter the first chemotherapy （ 18.2% vs 63.8% ,X2 =7. 828 ,P 〈0.01 ）. Although significant difference in death rate was not observed, EVIl （ ＋ ） group had significantly higher early-death rate （45.5% vs 8.6%, P 〈 0.01 ）. The EVIl （ ＋ ） group also had lower 4 years event-free survival （EFS） [ （21.2 ± 13.8 ） % vs （ 50.2 ± 9.1 ） % ,X2 = 4.493, P 〈 0.05 ] and lower 4 years overall survival（OS） [ （ 32.4 ± 7.1 ） % vs （ 60.3 ± 10.9 ） % ,X2 = 4. 602, P 〈 0.05 ] compared with EVIl （ - ） group. But binary Logistic analysis did not identify EVIl （ ＋ ） as an independent unfavorable prognostic fac- tor. Conclusions The pediatric AML with positive EVIl expression had lower CR rate,higher early death rate and lo- wer EFS. Positive EVIl expression is related with an adverse outcome, but is not an independent poor prognostic factor.

关 键 词：急性髓系白血病 EVI1基因 预后 儿童 急性髓系白血病 EVIl基因 预后 儿童 

分 类 号：R733.71[医药卫生—肿瘤]