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目的:探讨原发纵隔的弥漫大B细胞非霍奇金淋巴瘤(PMLBL)的临床特征及治疗策略。方法:回顾性分析34例原发纵隔的弥漫大B细胞非霍奇金淋巴瘤的临床特点和不同治疗方案对患者生存期的影响。结果:临床分期79.4％为Ⅰ-Ⅱ期,50.0％患者起病时出现上腔静脉压迫综合征,47.1％有邻近器官侵犯。采用化、放疗联合治疗29例(85.3％)、单纯化疗5例(14.7％)、自体造血干细胞移植9例(26.4％)。根据寿命表法分析,全组5和10年无瘤生存率(DFS)为47.8％和41.0％,5和10年总生存率(OS)为54.4％和54.4％。自体造血干细胞移植组较常规化、放疗组5年DFS和OS均有所提高,但无显著性差异(P>0.05)。不良预后因素分析显示,具有巨大肿块及治疗后来能获得完全缓解者预后差(P<0.05)。结论:原发纵隔的弥漫大B细胞非霍奇金淋巴瘤具有独特的临床和病理特点,治疗以化、放疗为主。具有不良预后因素的患者可考虑自体外周血造血干细胞移植(APBSCT),但最佳的治疗方案尚需进一步的前瞻性随机研究证实。
Objective: To investigate the clinical features and treatment strategies of primary mediastinal diffuse large B cell non-Hodgkin’s lymphoma (PMLBL). Methods: A retrospective analysis of 34 cases of primary mediastinal diffuse large B cell non-Hodgkin’s lymphoma clinical features and different treatment options on the survival of patients. Results: 79.4% of the patients were stage Ⅰ-Ⅱ, and 50.0% of the patients presented with superior vena cava syndrome at onset and 47.1% had adjacent organ invasion. 29 cases (85.3%) were treated with combined chemotherapy and radiotherapy, 5 cases (14.7%) were chemotherapy alone, and 9 cases (26.4%) were autologous stem cell transplantation. According to the life table analysis, the 5-year and 10-year disease-free survival rates (DFS) were 47.8% and 41.0%, respectively. The 5-year and 10-year overall survival rates were 54.4% and 54.4%, respectively. Compared with the conventional hematopoietic stem cell transplantation group, the 5-year DFS and OS of the radiotherapy group were improved, but there was no significant difference (P> 0.05). Adverse prognostic factor analysis revealed a poor prognosis for patients with huge lumps and complete remission after treatment (P <0.05). Conclusion: The primary mediastinal diffuse large B-cell non-Hodgkin’s lymphoma has unique clinical and pathological features. The treatment is mainly chemotherapy and radiotherapy. Patients with adverse prognostic factors may consider autologous peripheral blood stem cell transplantation (APBSCT), but the best treatment options need further prospective randomized studies confirmed.