间变性大细胞淋巴瘤患者骨髓及外周血NPM—ALK融合基因表达与预后的关系  

Prognostic significance of the NPM-ALK fusion gene in bone marrow and peripheral blood for patients with anaplastic large cell lymphoma

作　　者：杨菁[1] 赵晓曦[1] 金铃[1] 段彦龙[1] 黄爽[1] 张梦[1] 张蕊[1] 周春菊[1] 张永红[1] 

机构地区：[1]首都医科大学附属北京儿童医院血液病中心,100045

出　　处：《中华血液学杂志》2013年第8期700-703,共4页Chinese Journal of Hematology

基　　金：北京市卫生局首都医学发展科研基金（2007-1030）

摘　　要：目的探讨间变性大细胞淋巴瘤（ALcL）患者骨髓及外周血NPM-ALK融合基因的表达与预后的关系。方法应用RT-PCR法检测21例ALCL患者骨髓（21份标本）及外周血（15份标本）细胞NPM-ALK融合基因的表达，并对其与患者的预后及临床特征之间的关系进行统计学分析。结果21例患者中男12例，女9例，中位年龄9（2～14）岁。21例患者中位随访时间31个月。骨髓细胞NPM-ALK阳性患者3年无事件生存率为（35．6±18．6）％，阴性患者为（91．7±8．0）％，差异有统计学意义（P=0．038）。患者骨髓细胞NPM—ALK阳性与其有3个以上器官受累（P=0．032）有相关性。86．7％的患者外周血与骨髓NPM．ALK检测结果一致。结论通过RT-PCR法检测ALCL患者骨髓及外周血NPM-ALK融合基因表达，可以证实患者血循环中有无微小肿瘤细胞播散，阳性结果是重要的预后不良因素，对指导分层治疗有重要的临床意义。Objective To investigate the expression of NPM-ALK fusion gene in bone marrow （BM） and peripheral blood （PB） in anaplastic large cell lymphoma （ALCL） patients and its prognostic significance. Methods NPM-ALK fusion gene of 21 BM and 15 PB samples from patients with NPM- ALK positive ALCL was detected by RT-PCR, and the relationship between NPM-ALK expression and prognosis and clinical characters was evaluated. Results Of the 21 patients, 12 cases were male and 9 case were female with a median age of 9 （range, 2-14） years old. The median follow-up was 31months. Patients with a positive NPM-ALK expression in BM had a 3-years EFS of （35.6±18.6）%, compared with （91.7±8.0）% for patients with negative NPM-ALK （P-=0.038）. The incidence of positive expression in BM was significantly higher in patients who had more than 3 organs involved by tumor （P=0.032）. 86.7% patients had a concordant results of NPM-ALK expression in PB and BM. Conclusion We could evaluate the minimal disseminated disease of NPM-ALK positive ALCL patients by screening the NPM-ALK fusion gene in BM and PB by RT-PCR. The positive expression is associated with a poor prognosis and could be used for stratification of ALCL.

关 键 词：淋巴瘤 大细胞 间变性 儿童 逆转录聚合酶链反应 融合基因 NPM-ALK 预后 

分 类 号：R733.1[医药卫生—肿瘤]