儿童T淋巴母细胞淋巴瘤研究进展

摘要

Lymphoblastic lymphoma (LBL) is a rare aggressive neoplasm of T-/B-precursors resembling acute lymphoblastic leukemia,which develops very fast with high morbidity.T-lymphoblastic lymphoma (T-LBL),accounting for 85％-90％ of LBL,develops more frequently in children and young adults and is typically characterized by a grossly enlarged mediastinum,whose diagnostic hallmark is the expression of a T-precursor cell immunophenotype.Recently many new diagnostic technologies,such as polymerase chain reaction,flow cytometry,fluorescence in situ hybridization and so on,have been used for the dignosis of LBL,which lay the foundation of the precision therapy for LBL.The adoption of pediatric-derived,intensive lymphoblastic leukemia-like protocols leads to significantly improved results,with event free survival about 75％-90％ in children.New clinical trials will introduce and confirm the value of new drugs and targeting agents,which may have more good effects.New minimal residual disease assessment methods(eg,next generation sequencing,NGS) make it possible to detect MRD in different subtype of childhood lymphomas.Now,the new advances in diagnosis and treatment of T-LBL were reviewed.%淋巴母细胞淋巴瘤(LBL)是T/B前体细胞罕见高侵袭性肿瘤,进展快,病死率高,T-LBL占LBL的85％～90％,典型临床表现为纵隔肿块,儿童和青少年发生率高.目前,很多诊断新技术,如聚合酶链反应、免疫组织化学、流式细胞术、荧光原位杂交等,已用于淋巴瘤诊断,为淋巴瘤精准治疗奠定基础.正电子发射计算机断层显像在成人淋巴瘤诊断及分期中已有较多应用,而儿童甚少.采用ALL样化疗方案使LBL各年龄组治疗结果显著改善,儿童无事件生存率达75％～90％,是LBL治疗史上重大进展之一.临床试验将引进新药物以改善治疗效果.微小残留病变检测技术不断提高,使其在儿童淋巴瘤中应用成为可能.现主要就T-LBL诊治新进展进行综述.