摘要目的 分析儿童淋巴母细胞淋巴瘤(LBL)的临床特点,探讨其预后相关因素.方法 2003年1月至2009年12月间北京儿童医院血液病中心收治的112例LBL住院患儿中,男74例,女38例,男女之比为1.9∶1,中位发病年龄7.0岁.T细胞型LBL(T-LBL) 73例,B细胞型LBL(B-LBL)39例.临床Ⅱ期6例,Ⅲ期25例,Ⅳ期81例.所有患者均采用BCH-LBL-2003方案化疗.结果 全组中位随访时间29个月(1～90个月),在诱导缓解33 d和化疗3个月进行评估,骨髓的完全缓解(CR)率分别为96.4％和100％；瘤灶的CR率分别为77.7％和94.5％,部分缓解(PR)率分别为22.3％和5.5％,总有效率为100％.全组患者的3年总生存率为89.1％,5年总生存率为87.0％；3年无病生存率(EFS)为85.4％,5年EFS为83.3％.全组复发11例,其中中枢神经系统复发3例,骨髓复发4例,原发肿瘤部位复发3例,颈部淋巴结+骨髓复发1例.死亡11例,其中感染相关死亡3例,复发后进展死亡8例.化疗期间所有患者出现Ⅲ～Ⅳ级骨髓抑制.单因素分析显示,诱导结束时未达CR、巨大瘤块、T细胞型和病程＜30d为LBL不良预后因素.结论 儿童LBL为高度恶性非霍奇金淋巴瘤,BCH-LBL-2003方案的化疗疗效良好,化疗相关的毒副反应可耐受.诱导结束时未达CR、巨大瘤块、T细胞型和病程＜30d的患者可能预后不良,应在早期给予更有效的治疗.

abstractsObjective To evaluate the clinical characteristics of childhood lymphoblastic lymphoma (LBL) and therapeutic efficacy of BCH-LBL-2003 regimen modified from BFM-90 protocol.The drugrelated toxicities and prognostic factors were explored at the same time.Methods From Janurary 2003 to December 2009,112 newly diagnosed LBL patients at the Hematology Center of Beijing Children's Hospital were enrolled in this study.The patients were treated with modified BFM-90-LBL protocol.Results At a median follow-up of 29 months ( 1 to 90 months),the patients were evaluated on day 33 and at the end of induction therapy.The bone marrow complete remission (CR) rates were 96.4％ and 100％,respectively.Meanwhile,the complete remission (CR) rates of tumor were 77.7％ and 94.5％,and the partial remission (PR) rates were 22.3％ and 5.5％,respectively.The overall response rate was 100％.The 3-year overall survival (OS) rate was 89.1％ and 5-year OS was 87.0％.The 3-year event-free survival (EFS) was 85.4％ and 5-year EFS was 83.3％.Eleven cases relapsed during the treatment (4 BM relapses,3 CNS recurrences,3 primary site and 1 lymph node of neck and BM).Eleven patients died (3 died of infection and 8 died of progressive disease after relapse).All patients experienced grade 3-4 hematological toxicity.Univariate analysis indicated that lack of CR at the end of induction therapy,immunophenotype,bulky tumor and course of disease had prognostic significance.Conclusions Lymphoblastic lymphoma in childhood and adolescence is highly aggressive.BCH-LBL-2003 protocol is very effective.The treatment-associated side effects were tolerable. Patients who didn't get CR at the end of induction therapy,with T-cell immunophenotype,with bulky disease and the course of disease less than 30 days may have a poor prognosis.