Therapeutic advances in double hit and double expressor large B-cell lymphoma
陈欣楠 1高贝贝1
扫码查看
点击上方二维码区域，可以放大扫码查看
作者信息
1. 大连医科大学附属第二医院血液科辽宁省造血干细胞移植医学中心,辽宁大连 116027
折叠
摘要
双打击(double hit lymphoma,DHL)和双表达(double expressor lymphoma,DEL)淋巴瘤是弥漫大B细胞淋巴瘤的两种特殊亚型,与MYC、BCL2及BCL6的异常表达有关,具有独特的遗传学特征,且临床预后差,目前临床常用的标准化学免疫疗法如R-CHOP(利妥昔单抗、环磷酰胺、多柔比星、长春新碱、泼尼松)无法达到令人满意的疗效,国际上尚无统一的治疗标准.强化化疗方案如R-Hyper-CVAD(利妥昔单抗、环磷酰胺、长春新碱、多柔比星、地塞米松)、R-EPOCH(利妥昔单抗、依托泊苷、泼尼松、长春新碱、环磷酰胺、多柔比星)、R-ICE(利妥昔单抗、异环磷酰胺、卡泊、依托泊苷)等显示了良好的疗效,改善了这部分患者的预后.对于首次达到完全缓解的DHL/DEL推荐进行高剂量化疗联合自体造血干细胞移植巩固治疗,降低治疗后复发风险.复发/难治型DHL/DEL预后差,异基因造血干细胞移植可能是潜在的治愈方法,但需大样本临床研究进行验证.CD19靶向嵌合抗原受体(chimeric antigen receptor,CAR)T细胞治疗在DHL/DEL中显示了良好的疗效及持续缓解.DHL/DEL存在明确的基因异常,近年来针对肿瘤遗传学特征的研究为靶向治疗提供了依据,为制定更有针对性精准治疗策略至关重要,目前靶向药物的联合治疗显示了良好的治疗前景,是未来的研究方向.文章较为全面地对DHL和DEL的治疗进展进行了综述,为临床治疗提供参考.
Abstract
Double hit lymphoma(DHL)and double expressor lymphoma(DEL)are two specific subtypes of diffuse large B cell lymphoma(DLBCL),associated with aberrant expression of MYC,BCL-2 and BCL-6,featuring distinctive genetic characteristics and a poor clinical prognosis.The standard chemoimmunotherapies currently in clinical use,such as R-CHOP,fails to yield the satisfactory therapeutic effects,and there is no consensus on criteria for treatment.Intensive chemotherapy,such as R-Hyper-CVAD、R-EPOCH and R-ICE,have shown good efficacy and improved the prognosis of DHL and DEL patients.For patients who have achieved complete response(CR)for the first time,it is recommended to perform high-dose chemotherapy following autologous hematopoietic stem cell transplantation(auto-HSCT)as consolidative therapy to mitigate the risk of post-treatment relapse.Relapsed or refractory DHL/DEL typically has a dismal prognosis.Allogeneic hematopoietic stem cell transplantation may be a potential cure but needs to be validated in large clinical studies.CD19-directed chimeric antigen receptor(CAR)T-cell therapy has manifested commendable efficacy and continuous remission in DHL/DEL.Definite genetic abnormalities exist in DHL/DEL.In recent years,investigations about the genetic characteristics of tumors have offered a basis for targeted therapy and are of crucial importance for formulating more precise treatment strategies.Presently,the combined use of targeted drugs has demonstrated favorable therapeutic prospects and represents the research direction in the future.In this article,the treatment progress of DHL and DEL is more comprehensively reviewed to provide reference for clinical treatment.
关键词
双打击淋巴瘤/双表达淋巴瘤/造血干细胞移植/靶向治疗
Key words
Double hit lymphoma/Double expressor lymphoma/Hematopoietic stem cell transplantation/Targeted therapy
维普
万方数据