摘要
目的，总结我国儿童淋巴母细胞型非霍奇金淋巴瘤（LBL）的临床特征、治疗反应及转归，以期更好地规范治疗，进-步改善预后。方法2009年1月至2013年6月7个儿童肿瘤中心参与此项回顾性研究。以德国柏林-法兰克福-蒙斯特协组90／95方案（BFM-90／95）方案为基本框架，修改制定了上海儿童医学中心2007版方案（SCMC-2007）、上海儿童医学中心2011版方案（SCMC．2011）和中国儿童非霍奇金淋巴瘤2010版方案（NHL-2010）三个方案，其主要差别为阿霉素剂量、甲氨蝶呤剂量及鞘内化疗次数变化。其中，上海儿童医学中心采用SCMC-2007和SCMC．2011方案治疗，其他中心采用NHL-2010方案。以邮件通讯方式完成资料采集，分析我国LBL患儿临床特征和实验室检查特点，采用Kaplan—Meier方法计算LBL患儿总体生存率（OS）及无事件生存率（EFS），多因素Logistic回归分析LBL患儿进展或复发的危险因素。结果（1）96例患儿入组，发病年龄8．6（1．1～16．2）岁，男73例、女23例，男女比例为3．2：1；（2）T细胞型LBL77例（80％），B细胞型LBL19例（20％）。Ⅰ期6例（6％），Ⅱ期6例（6％），Ⅲ期51例（53％），Ⅳ期32例（33％），1例为孤立性骨病灶；（3）随访至2014年6月30日，随访时间为21．0（0．3—60．7）个月，2年OS为（89±3）％，EFS为（68±5）％；预期5年0s为（88±4）％，EFS为（64±6）％。其中，23例进入SCMC-2007治疗组，15例进入SCMC-2011治疗组，58例进入NHL-2010治疗组，2年OS分别为（91±6）％、100％、（83±6）％；2年EFS分别为（70±10）％、（92±8）％、（72±6）％。治疗第33天评估，病情缓解者2年EFS明显高于病情未缓解者，分别为（77±6）％和（17±10）％（P〈0．005）；（4）本组患儿13例复发，复发率为20％，复发时间8（1—31）个月，病情进展者6例。多因素Logistic回归分析发现治疗第33天病情未缓解是LBL患儿疾病进展或复发的危险因素，其OR值为4．138（P=0．035）。结论我国儿童LBL患儿诊治水平与国际上仍存在-定的差距，如何进-步提高我国LBL患儿的长期生存率，减少复发，还有待于-步修改治疗方案，加强国内各治疗中心的协作。
Objective To evaluate the feasibility of conducting a collaborative study and efficacy of lymphoblastic non-Hodgkin＇ s lymphoma （LBL） treatment protocols in children with LBL in China retrospectively and to explore the clinical and prognostic features as well as treatment response of LBL, in order to better regulate the treatment for further improving the prognosis. Method Seven tertiary referralcenters tor childhood cancer actively participated in this study. Shanghai Children＇s Medical Center （SCMC） 2007, SCMC-2011 and NHL-2010 protocols of treatment were modified based on BFM-90/95 regimen m＇iginated from Germany. SCMC-2007 and SCMC-2011 were used in Shanghai Children＇s Medical Center and NHL-2010 was used in other centers. Uniform data collection forms were sent out to all the centers and collected back via e-mail. The clinical data were then analyzed with primary focus on clinical presentations, and laboratory findings. Kaplan-Meier method was used to compare the survival rates between groups, while multiple factors logistic regression was used to identify the prognostic/relapse factors. Result （ 1 ） A total of 96 patients were recruited. The median age at diagnosis was 8.6 （ 1.1 - 16.2） years. The male-to-female ratio was 3.2： 1. （2） The immunophenotype was T-cell LBL in 77 patients （80%） and precursor B-cell LBL in 19 patients （20%）, respectively. According to St. Jude staging classification, 6 patients （6%） were divided into stage I , 6 patients （6%） into stage II, 51 patients （53%） into stage Ⅲ, 32 patients （33%） into stage Ⅳ and 1 patient with isolated bone involvement. （3） With a median follow up of 21 months （0. 3 -60. 7 months） , the 2-year overall survival （OS） rates and event-free survival （EFS） rates were （89 ±3）% and （68 ±5）% , respectively, and the 5-year probability of OS and EFS were （88 ±4）% and （64±6）%, respectively in all patients. Out of all, 23 patients received SCMC-2007 protocol, 15 patients received SCMC-2011 protocol and 58 patients received NHL-2010 protocol. The 2-year OS and EFS were （91±6）% and （70±10）% in SCMC-2007 study, 100% and （92 -＋8）% in SCMC-2011 study, （83 ± 6）% and （72 ± 6）% in NHL-2010 study, respectively. For patients with complete response on day 33, the 2-year EFS was （77 ± 6） % compared with （ 17 ±10） % for patients with incomplete response （ P 〈 0. 005）. （4） In 13 patients （20%） the disease relapsed. The median relapse time range was 8 months （1 -31 months）. Six patients suitbred from progressive disease. Logistic regression analysis showed that incomplete response on days 33 was an independent unfavorable prognostic factor （ OR = 4. 138, P = 0. 035 ）. Conclusion Despite the apparent existence of gap between China and International states in the diagnostic and treatment approaches towards childhood LBL in China, with implementation of＇ further modification of treatment protocols to strengthen cooperation between domestic tertimy centers, the long-term survival rate and reduction of recurrence of childhood LBL in China can be markedly improved.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2015年第12期931-937,共7页
Chinese Journal of Pediatrics
