摘要目的 探讨儿童成熟B细胞非霍奇金淋巴瘤(B-NHL)诊治方案(CCCG-B-NHL 2010)的疗效,为进一步改善多中心协作管理模式和方案改进提供依据.方法 8个医疗单位参与此项回顾性研究.收集2009年4月1日至2012年3月31日收治的年龄≤18岁的初治B-NHL和成熟B细胞型急性淋巴细胞白血病(B-ALL)病例,最后随访时间为2014年1月31日.104例患儿均按照CCCG-B-NHL 2010方案进行治疗.其中23例患儿在CCCG-B-NHL 2010基础上联合利妥昔单抗治疗.分析患儿临床特征、治疗结果,并应用Kaplan-Meier法对患儿进行生存分析.结果 104例患儿中男79例,女25例,中位年龄6.1岁(1.8～15.1岁).其中Ⅰ期2例,Ⅱ期22例,Ⅲ期65例,Ⅳ期9例,B-ALL 6例.单纯化疗组(81例)和联合利妥昔单抗组(23例)在疾病分期和乳酸脱氢酶(LDH)水平分布上差异均无统计学意义(x2 =1.44和3.99,P均＞0.05).本组6例患儿病情未缓解并进展至死亡,12例复发,其中11例复发时间6.9个月(3.1 ～23.4个月),另1例于34.9个月复发,不能除外第二肿瘤.本组放弃治疗率为10.9％ (12/110),治疗相关死亡率1％ (1/104).中位随访时间为27.9个月(4.2 ～51.5个月),2年无事件生存率(EFS)为(76.0±4.3)％.联合利妥昔单抗治疗组和单纯化疗组的2年EFS差异无统计学意义[分别为(76.1±4.8)％和(75.9±9.6)％,P＞0.05].LDH＜正常值2倍,2～4倍和＞4倍患儿的2年EFS分别为(84.5±4.8)％、(70.6±11.1)％、(58.0±10.1)％(P=0.02).Ⅰ、Ⅱ、Ⅲ以及Ⅳ期/B-ALL患儿的2年EFS分别为100％、(93.3±6.4)％、(75.1±5.4)％和(52.5±13.1)％(P=0.03).结论 国内多中心研究需进一步建立病例分期、分组及病理中央复核制度,并推行淋巴瘤登记制度.提高LDH＞正常值4倍、Ⅳ期和B-ALL晚期患儿的生存率是下一期方案研究的重点.

abstractsObjective This study aimed to evaluate the efficacy of the CCCG-B-NHL 2010 protocol in children with mature B-cell non-Hodgkin's lymphoma (B-NHL) in China retrospectively.Method Eight tertiary referral centers for childhood cancer participated in this study.From April 2009 to March 2012,104patients below 18 years with newly diagnosed,untreated B-NHL or mature B cell acute lymphoblastic leukemia (B-ALL) were enrolled.Six patients refused further staging work-up and treatment due to the expense were excluded.Diagnostic slides were not centrally reviewed in this retrospective study.Twentythree of 104 patients got rituximab therapy during the treatment.Result Of the 104 eligible patients (79boys and 25 girls),the median age was 6.1 years (range 1.8-15.1 years).Two patients (1.9％) had stage Ⅰ disease,22 (21.2％) had stage Ⅱ,65 (62.5％) had stage Ⅲ,9 (8.6％) had stage Ⅳ,and 6 (5.8％) had B-ALL.At a median follow-up of 27.9 months (range 4.2-51.5 months),the 2-year probability of event-free survival (EFS) was (76.0 ±4.3)％ in all patients.The 2-year EFS was 100％,(93.3-± 6.4) ％,(75.1 ± 5.4) ％ and (52.5 ± 13.1) ％ for patients with stage Ⅰ,Ⅱ,Ⅲ and Ⅳ/ BALL,respectively (P =0.03).There was no significant difference in EFS between patients treated with chemotherapy only and those with chemotherapy combined with rituximab [(76.1 ± 4.8) ％ vs.(75.9 ±9.6)％,P＞0.05].The2-yearEFS was (84.5±4.8)％,(70.6±11.1)％ and (58.0±10.1)％ for patients with LDH ＜ 2 times the institutional upper limit of normal (2 times NL),2-4 times NL and ＞4 times NL,respectively (P =0.02).Only one patient (1％,1/104) died of treatment-related complications.Six patients refused treatment during therapy.Including the 6 cases who refused to receive further staging diagnostic workup,the abandonment rate for B-NHL/B-ALL was 10.9％ (12/110).Conclusion The EFS rate in this study was much lower in patients with advanced disease (LDH ＞ 4 times NL,stage Ⅳ and B-ALL) than that of series conducted in medically developed countries.Establishment of strategies to improve survival in patient with advanced disease is the priorities in our following study.