Objective: To analyse the long-term efficacy in childhood T-cell acute lymphoblastic leukemia (T-ALL) cases enrolled in the national protocol of childhood leukemia in China-acute lymphoblastic leukemia (NPCLC-ALL) 2008. Methods: Clinical data of 96 patients diagnosed as T-ALL and treated with NPCLC-ALL2008 protocol between January 2009 and December 2017 in the Department of Hematology-Oncology, the Children's Hospital, Zhejiang University School of Medicine were analyzed retrospectively. Predictive value of minimal residual disease (MRD) monitored by flow cytometry was analyzed. Kaplan-Meier method was used for long-term survival analysis. Results: A total of 96 evaluable patients with newly diagnosed T-ALL were analysed, including 72 males and 24 females. The age was 9.5 (ranged from 1.0 to 16.0) years. The follow-up time was 5.7 (ranged from 1.0 to 9.7) years. Among 96 patients, 92 (96%) achieved complete remission. The 5-year event free survival (EFS) and overall survival (OS) rates were (61±6) % and (70±5) %, respectively. Relapse occurred in 18 cases and the 5-year cumulative incidence of relapse was (27±6) %. Twenty-four patients died. The 5-year OS rates of patients with MRD>5% on day 15 of induction therapy was significantly worse than those with MRD≤5% ((60±12) % vs. (72±6) %, χ(2)=3.904, P=0.048) . The 5-year EFS and OS rates were obviously lower in patients with MRD>10% before the consolidation therapy ((50±35) %). The 5-year OS rates of patients with relapsed disease was significantly worse than those without ((26±13) % vs. (81±5) %, χ(2)=18.411, P<0.01). The earlier the relapse, the worse the prognosis. The 5-year OS rates for patients relapsed within 6 months, within 3 years and more than 3 years, were (25±22) %, (30±14) % and (50±35) % respectively (χ(2)=13.207, P<0.01). Conclusions: NPCLC-ALL2008 protocol is effective for childhood T-ALL. The MRD guided accurate risk stratification and individualized treatment can reduce the relapse and improve the survival rate of pediatric T-ALL.
目的： 探讨中国儿童急性淋巴细胞白血病2008（NPCLC-ALL2008）方案治疗儿童急性T淋巴细胞白血病（T-ALL）的效果。 方法： 回顾性分析2009年1月至2017年12月在浙江大学医学院附属儿童医院血液肿瘤中心接受NPCLC-ALL2008方案进行规范治疗的96例T-ALL患儿的临床资料。分析流式细胞术监测微小残留病（MRD）与预后的关系，应用Kaplan-Meier法进行长期生存分析。 结果： 96例T-ALL患儿中男72例、女24例，年龄9.5（1.0~16.0）岁，随访时间5.7（1.0~9.7）年。92例患儿获得完全缓解（96%），5年无事件生存率（EFS）和总生存率（OS）分别为（61±6）%和（70±5）%，复发18例，5年累积复发率（27±6）%，死亡24例。诱导第15天MRD>5%者生存率明显低于MRD≤5%者，5年OS分别为（60±12）%和（72±6）%（χ(2)=3.904, P=0.048），巩固治疗前MRD>10%者5年EFS和OS均明显降低，均为（50±35）%。复发患儿生存明显差于无复发患儿，5年OS分别为（26±13）%和（81±5）%（χ(2)=18.411, P<0.01），复发时间越早预后越差，诊断6个月内复发者5年OS仅为（25±22）%，3年内和3年以上复发者5年OS分别为（30±14）%和（50±35）%，差异有统计学意义（χ(2)=13.207, P<0.01）。 结论： NPCLC-ALL2008方案治疗T-ALL疗效尚可，MRD指导下进行精确的危险分层和个性化治疗来减少复发有望进一步提高T-ALL患儿长期生存率。.
Keywords: Child; Leukemia, T-cell; Prognosis; Recurrence.