Objective: To investigate the impact of intensified maintenance therapy on the prognosis of children and adolescents with advanced lymphoblastic lymphoma (LBL) . Methods: Retrospective analysis on the treatment results of children and adolescents with stage Ⅲ and stage Ⅳ LBL who underwent BFM-NHL-90/-95 regimen without prophylactic radiotherapy. The intensified therapy group included the patients admitted from 1998 to 2005, while others were classified as the non-intensified therapy group. Patients in the intensified therapy group were intravenously treated with "etoposide phosphate plus cytrarabine" and high-dose methotrexate alternately per 2.5-3 months in addition to the oral chemotherapy with 6-mercaptopurine and methotrexate during the maintenance phase. Results: A total of 187 LBL patients were enrolled. The rates of 5-year event free survival were (76.9 ± 5.8) % and (77.9 ± 4.3) % (χ(2)=0.249, P=0.617) respectively, in the intensified therapy (n=52) and the non-intensified therapy groups (n=135) , while the rates of 5-year overall survival of them were (78.8 ± 5.7) % and (79.8±4.1) % (χ(2)=0.353, P=0.552) , respectively. Stratified by stage, immunological type as well as risk stratification, the rates of long-term survival were similar between the two groups. During the maintenance phase, the rates of grade Ⅲ and Ⅳ myelosuppression in the intensified therapy and the non-intensified maintenance groups were 55.8% and 18.5%, respectively (χ(2)=25.363, P<0.05) . Conclusion: Intensified maintenance therapy failed to improve the prognosis of patients with advanced LBL.
目的： 探讨增加维持治疗强度对Ⅲ期和Ⅳ期儿童青少年淋巴母细胞淋巴瘤（Lymphoblastic lymphoma, LBL）患者预后的影响。 方法： 回顾性分析接受BFM-NHL-90/-95方案治疗且未做纵隔和中枢预防性放疗的Ⅲ期和Ⅳ期儿童青少年LBL患者的治疗结果。研究分组：1998年至2005年收治的患者于维持治疗阶段，在口服巯基嘌呤和甲氨蝶呤的基础上，定期采用"足叶乙甙+阿糖胞苷"和大剂量甲氨蝶呤交替进行化疗，为强化维持治疗组；其余为非强化维持治疗组。 结果： 187例LBL患者纳入研究，其中强化维持治疗组52例，非强化维持治疗组135例，两组患者的性别、年龄、免疫分型、临床分期、危险度分层、受累部位的差异均无统计学意义（P值均>0.05）；中位随访48（0.5～221）个月，两组患者的5年无事件生存（EFS）率分别为（76.9±5.8）%和（77.9±4.3）%（χ(2)＝0.249，P＝0.617），5年总生存（OS）率分别为（78.8±5.7）%和（79.8±4.1）%（χ(2)＝0.353，P＝0.552），差异均无统计学意义；亚组分析结果显示，两组患者在不同临床分期（Ⅲ/Ⅳ期）、免疫分型（T/B-LBL）和危险分层（中/高危）中的EFS、OS率差异均无统计学意义（P值均>0.05）。维持治疗期间，强化维持治疗组和非强化维持治疗组患者Ⅲ、Ⅳ级骨髓抑制发生率分别为55.8%和18.5%（χ(2)＝25.363，P<0.05）。 结论： 提高维持治疗强度并未提高Ⅲ期和Ⅳ期儿童青少年LBL患者的长期生存且可增加骨髓抑制等治疗相关不良反应。.
Keywords: Adolescent; Children; Maintenance chemotherapy; Precursor cell lymphoblastic leukemia-lymphoma; Prognosis.