多中心治疗儿童急性T淋巴细胞性白血病的长期疗效评价及随访报告

Evaluation of long-term therapeutic effects of children with T cell acute lymphoblastic leukemia of multiple medical centers and the follow-up report

目的评估急性淋巴细胞白血病(ALL)-2005方案对儿童T淋巴细胞白血病(T-ALL)的临床疗效。方法收集源自5所儿童血液病治疗中心的2005年5月1日—2009年9月30日初次发病,并纳入ALL-2005方案治疗的53例T-ALL患儿的临床资料,患儿分别按照中危和高危进行分组后接受不同强度的化疗。回顾性分析诱导缓解率、治疗相关并发症及随访情况,采用Kaplan-Meier生存分析法对T-ALL预后的影响因素进行分析。结果 53例T-ALL患儿,剔除1例患儿(确诊后2 d因弥散性血管内凝血死亡),男44例,女8例;诊断中位年龄为8.1岁,年龄≥10岁的患儿16例(19.2%);外周血白细胞计数≥50×109/L的患儿35例(67.3%)。治疗后第35日的诱导缓解率为88.5%;复发病例为7例(13.5%),复发中位时间为8.3个月,其中骨髓复发4例,中枢神经系统复发3例。随访中位时间为62.0个月;8年总体生存率为(82.6±5.2)%,其中中危组为(91.1±3.2)%,高危组为(51.5±13.7)%,两组间差异有统计学意义(P<0.000 1);8年无事件生存率为(68.4±6.1)%,中危组和高危组分别为(74.6±5.8)%和(36.9±12.9)%,两组间差异有统计学意义(P<0.000 1)。结论 ALL-2005方案治疗儿童T-ALL可获得较好疗效。诱导期是否获得完全缓解以及危险度分组是影响预后的独立因素。

Objective To evaluate the clinical effects acute lymphoblastic leukemia （ T-ALL）. Methods of ALL-2005 protocol on children with T lymphocyte Clinical data of 53 patients with T-ALL from five Children＇s Medical Centers of Hematology/Oncology between 1st May 2005 and 30th September 2009 were selected and treated by the ALL-2005 protocol. All patients underwent the chemotherapy of different dose intensity according to their risk levels （ medium risk or high risk）. The remission rate of induction, complications relevant to the treatment, and follow-up results were retrospectively analyzed. Factors influencing the prognosis of T-ALL were analyzed by the Kaplan-Meier survival analysis. Results Among 52 patients （ excluding one patient who died of disseminated intravascular coagulation two days after diagnosis）, 44 of them were males and 8 were females. The median age of diagnosis was 8.1 years old and 16 cases （19.2%） were over 10 years old. The white blood cell count of 35 patients （67.3%） was higher than or equal to 50 × 109/L. The remission rate of induction was 88.5% on the 35th day after treatment. Seven cases （13.5%） relapsed and the median relapse time was 8.3 months. Among 7 cases, 4 cases suffered from bone marrow relapse and 3 cases suffered from central nervous system relapse. The median follow-up time was 62.0 months. The 8-year overall survival rates of all patients, medium risk group, and high risk group were （82.6 ±5.2） %, （91.1 ±3.2） % and （51.5 ± 13.7） %. The difference of two groups was statistically significant （P〈0. 000 1）. The 8-year event-free survival rates of all patients, medium risk group, and high risk group were （68.4 ±6.1） %, （74.6 ±5.8） %, and （36.9 ±12.9） %. The difference of two groups was statistically significant （P〈0. 0001）. Conclusion ALL-2005 protocol achieves good clinical outcome for children with T-ALL. Complete remission during the induction period and risk-based grouping are independent factors that influence the prognosis of T-ALL.