表达PRAME基因的儿童急性B淋巴细胞白血病的临床特点及预后分析  

Clinical features and prognosis of childhood B-lineage acute lymphoblastic leukemia expressing the PRAME gene

作　　者：张枫 陆爱东[1] 左英熹[1] 丁明明[1] 贾月萍[1] 张乐萍[1] ZHANG Feng;LU Ai-Dong;ZUO Ying-Xi;DING Ming-Ming;JIA Yue-Ping;ZHANG Le-Ping(Department of Pediatrics,People's Hospital,Peking University,Beijing 100044,China)

机构地区：[1]北京大学人民医院儿科,北京100044

出　　处：《中国当代儿科杂志》2022年第5期543-549,共7页Chinese Journal of Contemporary Pediatrics

基　　金：北京市临床重点专科项目(2018)。

摘　　要：目的研究在无特异性融合基因表达时,黑色素瘤特异性抗原(preferentially expressed antigen of melanoma,PRAME)基因阳性在儿童急性B淋巴细胞白血病(acute B lymphoblastic leukemia,B-ALL)中的临床及预后意义。方法纳入167例新诊断的B-ALL患儿,其中70例PRAME基因阳性,97例PRAME基因阴性,所有患儿均不表达MLL-r、BCR/ABL、E2A/PBX1、ETV6/RUNX1,分析2组患儿的临床特点、预后及预后的相关因素。结果PRAME阳性组肝肋下>6 cm患儿比例高于PRAME阴性组(P<0.05)。诱导化疗后PRAME基因拷贝数较治疗前下降(P<0.05),诱导化疗后微小残留病(minimal residual disease,MRD)阳性组中,PRAME基因拷贝数与MRD水平无相关性(P>0.05);在诱导化疗后MRD阴性组中,二者亦无相关性(P>0.05)。PRAME阳性组4年无事件生存率高于PRAME阴性组(87.5%±4.6%vs 73.5%±4.6%,P<0.05),2组4年总生存率差异无统计学意义(88.0%±4.4%vs 85.3%±3.8%,P>0.05)。Cox比例风险回归模型分析显示PRAME基因表达是影响B-ALL患儿4年无事件生存率的保护因素(P<0.05)。结论尽管PRAME基因不能作为MRD监测,但在B-ALL中PRAME基因过表达提示预后良好。Objective To study the clinical and prognostic significance of the preferentially expressed antigen of melanoma(PRAME)gene in the absence of specific fusion gene expression in children with B-lineage acute lymphoblastic leukemia(B-ALL).Methods A total of 167 children newly diagnosed with B-ALL were enrolled,among whom 70 were positive for the PRAME gene and 97 were negative.None of the children were positive for MLL-r,BCR/ABL,E2A/PBX1,or ETV6/RUNX1.The PRAME positive and negative groups were analyzed in terms of clinical features,prognosis,and related prognostic factors.Results Compared with the PRAME negative group,the PRAME positive group had a significantly higher proportion of children with the liver extending>6 cm below the costal margin(P<0.05).There was a significant reduction in the PRAME copy number after induction chemotherapy(P<0.05).In the minimal residual disease(MRD)positive group after induction chemotherapy,the PRAME copy number was not correlated with the MRD level(P>0.05).In the MRD negative group,there was also no correlation between them(P>0.05).The PRAME positive group had a significantly higher 4-year event-free survival rate than the PRAME negative group(87.5%±4.6%vs 73.5%±4.6%,P<0.05),while there was no significant difference between the two groups in the 4-year overall survival rate(88.0%±4.4%vs85.3%±3.8%,P>0.05).The Cox proportional-hazards regression model analysis showed that positive PRAME expression was a protective factor for event-free survival rate in children with B-ALL(P<0.05).Conclusions Although the PRAME gene cannot be monitored as MRD,overexpression of PRAME suggests a good prognosis in B-ALL.

关 键 词：急性淋巴细胞白血病 黑色素瘤特异性抗原 微小残留病 儿童 

分 类 号：R733.71[医药卫生—肿瘤]