携带SET-CAN融合基因儿童前T淋巴母细胞淋巴瘤1例并文献复习  

Pediatric T-lymphoblastic lymphoma with SET-CAN fusion gene: a case report and review of the literatures

作　　者：郜慧芳[1] 李伟京[1] 张永红[1] 李志刚[1] 金玲[1] 杨菁[1] 段彦龙[1] 黄爽[1] 张蕊[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,北京100045

出　　处：《中国小儿血液与肿瘤杂志》2011年第2期74-77,共4页Journal of China Pediatric Blood and Cancer

基　　金：北京市卫生局首都医学发展基金(2007-1030);北京市科技新星计划(2008B65)资助

摘　　要：目的探讨携带SET-CAN融合基因的儿童前T淋巴母细胞淋巴瘤的临床特点及该基因在血液恶性肿瘤发病中的可能机制。方法分析1例携带SET-CAN融合基因的儿童前T淋巴母细胞淋巴瘤的临床特点、治疗和预后情况,并对相关文献进行复习。结果本例患儿起病急,病情进展快,很快发生骨髓转移,受累范围广泛。按BCH-2003-LBL方案化疗,2周后骨髓完全缓解,化疗后第33天瘤灶消失,SET-CAN融合基因转为阴性,目前已维持治疗1年半,以SET-CAN融合基因做为MRD监测标志持续阴性。结论携带SET-CAN融合基因的前T淋巴母细胞淋巴瘤对化疗敏感,该基因在化疗后缓解期持续阴性,可能是预后良好的指标,SET-CAN融合基因可能成为监测淋巴瘤微小残留病的新标志。Objective To explore the clinical characteristics of pediatric T-lymphoblastic lymphoma（T-LBL） with SET-CAN fusion gene and expound the potential mechanism of SET-CAN in haematopoietic malignancy.Methods Clinical characteristics,treatment and outcome of a T-LBL case were analyzed.Literatures related to SET-CAN were reviewed.Results The onset of the disease was acute.It progressed very quickly and involved the bone marrow early.The involvement was extensive.The patient was treated with BCH-2003-LBL protocol and got complete bone marrow remission 2 weeks after chemotherapy.The tumor mass disappeared and SET-CAN gene turned negative at the day of 33.Now the patient was in maintenance therapy.SET-CAN gene monitored as MRD marker kept negative during 1 and a half years maintenance treatment.Conclusion T-LBL with SET-CAN fusion gene was sensitive to chemotherapy.SET-CAN gene kept negative in remission after chemotherapy.It implied that SET-CAN gene might be a favorable prognostic factor and a new MRD marker of Lymphoblastic lymphoma.

关 键 词：儿童 前T淋巴母细胞淋巴瘤 SET-CAN融合基因 

分 类 号：R733.1[医药卫生—肿瘤]