T淋巴母细胞淋巴瘤的诊治与预后影响因素的研究现状

Current research status of diagnosis,treatment and prognostic factors of T-lymphoblastic lymphoma

T淋巴母细胞淋巴瘤(T-LBL)为一类罕见的、具有高度侵袭性的淋巴瘤,多见于儿童及青少年男性,患者预后差,主要临床症状为纵隔肿块、淋巴结大、骨髓侵犯与中枢神经系统(CNS)受累等。T-LBL诊断主要根据淋巴结活组织检测、骨髓细胞形态学、免疫学、细胞遗传学及基因检测结果,确诊后应给予高剂量化疗药物的联合化疗及序贯造血干细胞移植(HSCT)或者维持治疗。影响该病患者预后的因素较多,包括年龄、骨髓受累、CNS受累、疾病分期、相关基因突变等。笔者拟就T-LBL的诊治及相关预后影响因素的最新研究进展进行综述,旨在为T-LBL的临床诊治提供参考。

T-lymphoblastic lymphoma(T-LBL) is a rare and highly aggressive lymphoma, with poor prognosis. It occurs mostly in children and male adolescents, and its main symptoms are mediastinal mass, lymphadenectasis, bone marrow invasion and central nervous system(CNS) invasion, etc.. The diagnosis of T-LBL is mainly based on lymph node biopsy, bone marrow cell morphology, immunology, cytogenetics and gene detection. After definitive diagnosis of T-LBL, the patients should be treated with extensive combined chemotherapy and sequential hematopoietic stem cell transplantation (HSCT) or maintenance treatment. There are many factors influencing the prognosis of T-LBL patients, such as age, bone marrow invasion, CNS invasion, disease stage and gene mutations. This article intends to introduce the development of diagnosis, therapy and prognostic factors of T-LBL to provide reference for the diagnosis and therapy of T-LBL.