70例儿童淋巴母细胞淋巴瘤远期疗效评估  

Long-term outcomes of childhood lymphoblastic lymphoma： report of 70 cases

作　　者：徐舟[1] 潘慈[1] 陈静[1] 薛惠良[1] 叶启东[1] 周敏[1] 罗长缨[1] 江华[1] 李本尚[1] 汤燕静[1] 王坚敏[1] 董璐[1] 汤静燕[1] 

机构地区：[1]上海交通大学附属上海儿童医学中心,200127

出　　处：《中华血液学杂志》2013年第12期1044-1049,共6页Chinese Journal of Hematology

摘　　要：目的总结儿童淋巴母细胞淋巴瘤（LBL）诊断治疗方案的远期疗效及临床特征。方法前瞻性制订诊断治疗方案，连续性积累病例，分析总结1998年11月至2010年10月收治的70例儿童LBL资料，其中T细胞型65例，B细胞型5例。22例采用CCCG-97方案化疗，48例采用CCCG-2002方案化疗。参照St．Jude分期标准进行分期，将患儿分为高、中、低危3组，根据分组分别给予不同强度的治疗方案。单因素分析法分析可能与预后相关的因素。结果70例患儿诊断时中位年龄8（1．5-14）岁，确诊时I-Ⅱ期6例，Ⅲ期41例，Ⅳ期23例（伴骨髓浸润15例，伴多发性骨转移8例）。随访至2011年12月31日，无病生存者平均随访时间62．5（14～161）个月，中位随访时间48个月。1年生存率74.3％；累计5年无事件生存率64．1％；化疗期间13例患儿出现严重不良反应，其中8例为明确病原菌的脓毒血症，4例为需要重症监护治疗的严重感染，化疗不良反应相关死亡1例。Kaplan—Meier法绘制生存曲线最示早期缓解（化疗第33天评估）及诱导完成后缓解情况与预后显著相关（P值均〈0．05）。结论儿童LBL纵隔原发多见，确诊时90％的患儿为Ⅲ～Ⅳ期，本组患儿5年无事件生存率为64．1％，治疗早期反应与远期预后相关。Objective To summarize long-term outcomes of childhood lymphoblastic lymphoma （LBL） with protocol CCCG-97 and-2002. Methods From November 1998 to October 2010, 70 consecutive newly diagnosed childhood LBL （5 B-LBL and 65 T-LBL） were enrolled in this study, in which 22 received CCCG-97 and 48 CCCG-2002 protocols. St.Jude staging system was adopted. Patients were divided into three risk groups based on clinical stage and serum LDH, and received chemotherapy with different intensity. The factors, which were possibly associated with the prognosis, were analyzed. The survival rates were evaluated by Kaplan-Meier analysis. Results The patients were 1.5 to 14 years old with the median age of 8 years old. They were evaluated as stage I - 1I for 6, stage [1141, and stage IV23 （ 15 were BM positive and 8 multiple bone metastases）. Until Dec.31 th, 2011, the mean follow-up was 62.5 months （range,14 to 161 months） with the median follow-up of 48 months. 1-year overall survival （OS） was 74.3%, and 5-year event-free survival （EFS） 64.1% （abundance as event）. Thirteen patients were complicated with serious condition during chemotherapy and 1 died of complication. Univariate analysis indicated that delayed and/or non-completed response on days 33 and 63 of induction was the unfavorable prognostic factor. Conclusion Primary LBL usually located in the mediastinum. 90% of the patients was at advanced stage HI-IV at first presentation. The 5-year EFS was 64.1%. Patients not achieved CR at days 33 and 63 at the end of induction was a poor prognostic factor.

关 键 词：前体细胞淋巴母细胞白血病淋巴瘤 儿童 抗肿瘤联合化疗方案 预后 

分 类 号：R733.1[医药卫生—肿瘤]