Zhang Yan-Huan, Lu Ai-Dong, Yang Lu, Li Ling-Di, Chen Wen-Min, Long Ling-Yu, Zhang Le-Ping, Qin Ya-Zhen
Peking University People's Hospital, Peking University Institute of Hematology, Beijing, China.
Peking University People's Hospital, Department of Pediatrics, Beijing, China.
Leuk Res. 2017 Jan;52:43-49. doi: 10.1016/j.leukres.2016.11.005. Epub 2016 Nov 11.
To investigate the prognostic value of PRAME expression in pediatric acute lymphoblastic leukemia(ALL), we measured PRAME transcript levels at diagnosis in 191 patients(146 B-ALL; 45T-ALL)receiving chemotherapy only. PRAME overexpression was defined as transcript levels higher than 0.30%, which is the upper limit of normal bone marrow and the optimal cutoff value derived from ROC curve analysis. PRAME overexpression was identified in 45.5% of patients. In B-ALL, PRAME overexpression was significantly associated with lower CIR(cumulative incidence of relapse), higher DFS (disease-freesurvival), and OS(overall survival) rates at 3 years, respectively (5.8% vs. 14.9%, P=0.014; 94.2% vs. 85.1%, P=0.014; 96.0% vs. 87.4%, P=0.039). PRAME overexpression had no impact on outcome in T-ALL patients. Among B-ALL patients with non-poor cytogenetic risk, those with PRAME overexpression showed significantly lower CIR, higher DFS and OS rates at 3 years, respectively (8.47% vs. 14.5%, P=0.009; 96.5% vs. 85.5%, P=0.009; 98.4% vs. 88.0%, P=0.023). Furthermore, PRAME overexpression was an independent good prognostic factor for relapse in all B-ALL patients and B-ALL patients with non-poor cytogenetic risk. Therefore, the prognostic significance of PRAME overexpression differed by ALL subtype; It predicted good outcome in pediatric B-ALL receiving chemotherapy.
为研究PRAME表达在儿童急性淋巴细胞白血病(ALL)中的预后价值,我们检测了191例仅接受化疗的患者(146例B-ALL;45例T-ALL)诊断时的PRAME转录水平。PRAME过表达定义为转录水平高于0.30%,这是正常骨髓的上限以及通过ROC曲线分析得出的最佳截断值。45.5%的患者存在PRAME过表达。在B-ALL中,PRAME过表达分别与3年时较低的复发累积发生率(CIR)、较高的无病生存率(DFS)和总生存率(OS)显著相关(5.8%对14.9%,P = 0.014;94.2%对85.1%,P = 0.014;96.0%对87.4%,P = 0.039)。PRAME过表达对T-ALL患者的预后无影响。在细胞遗传学风险非高危的B-ALL患者中,PRAME过表达的患者3年时的CIR、DFS和OS率分别显著较低(8.47%对14.5%,P = 0.009;96.5%对85.5%,P = 0.009;98.4%对88.0%,P = 0.023)。此外,PRAME过表达是所有B-ALL患者以及细胞遗传学风险非高危的B-ALL患者复发的独立良好预后因素。因此,PRAME过表达的预后意义因ALL亚型而异;它预示着接受化疗的儿童B-ALL预后良好。