表达<italic>PRAME</italic>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

doi:10.7499/j.issn.1008-8830.2111108

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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。引用格式：中国当代儿科杂志，2022，24（5）：543-549

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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

Abstract：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% vs 85.3%±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. Citation:Chinese Journal of Contemporary Pediatrics, 2022, 24(5): 543-549

Key words： Acute lymphoblastic leukemia Preferentially expressed antigen of melanoma Minimal residual disease Child

收稿日期: 2021-11-18

基金资助:北京市临床重点专科项目（2018）。

通讯作者: 张乐萍，女，主任医师。Email：zhangleping@pkuph.edu.cn。 E-mail: zhangleping@pkuph.edu.cn

作者简介: 张枫，女，硕士，主治医；

引用本文:

张枫,陆爱东,左英熹等. 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[J]. 中国当代儿科杂志, 2022, 24(5): 543-549.

ZHANG Feng,LU Ai-Dong,ZUO Ying-Xi et al. Clinical features and prognosis of childhood B-lineage acute lymphoblastic leukemia expressing the PRAME gene[J]. CJCP, 2022, 24(5): 543-549.

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