儿童T淋巴母细胞淋巴瘤采用白血病方案治疗的预后分析  

Prognostic analysis of childhood T-lymphoblastic lymphoma treated with leukemia regimen

作　　者：后书敏 邵静波[1] 李红[1] 张娜 朱嘉莳 王丹[1] 付盼 HOU Shu-Min;SHAO Jing-Bo;LI Hong;ZHANG Na;ZHU Jia-Shi;WANG Dan;FU Pan(Department of Hematology/Oncology,Shanghai Children's Hospital,School of Medicine,Shanghai Jiao Tong University,Shanghai 200040,China)

机构地区：[1]上海市儿童医院/上海交通大学医学院附属儿童医院血液肿瘤科,上海200040

出　　处：《中国当代儿科杂志》2024年第5期469-475,共7页Chinese Journal of Contemporary Pediatrics

基　　金：上海市科学技术委员会(23015820600);上海市卫生健康委员会科研课题青年项目(20204Y0471)。

摘　　要：目的探讨儿童T淋巴母细胞淋巴瘤(T-lymphoblastic lymphoma,T-LBL)采用急性淋巴细胞白血病(autelymphoblasticleukemia,ALL)方案治疗的预后及相关影响因素。方法回顾性总结2010年5月—2022年5月收治的29例T-LBL患儿采用同期ALL方案(ALL-2009方案或CCCG-ALL-2015方案)治疗的预后特点。结果29例T-LBL患儿5年总生存(overall survival rate,OS)率及无事件生存(event-free survival,EFS)率分别为(84±7)%、(81±8)%。初诊时全身症状有B症状(不明原因发热>38℃,连续3d以上;盗汗;体重在6个月内下降大于10%)的患儿5年EFS率低于无B症状患儿(P<0.05)。初诊时血小板计数>400×10^(9)/L、同时累及纵隔和淋巴结的患儿5年OS率较低(P<0.05)。CCCG-ALL-2015方案和ALL-2009方案的5年OS率及EFS率比较差异无统计学意义(P>0.05)。CCCG-ALL-2015方案相较于ALL-2009方案减少大剂量氨甲蝶呤化疗次数,降低重症感染率(P<0.05)。结论儿童T-LBL采用ALL方案治疗安全有效;初诊时全身症状有B症状、血小板计数>400×10^(9)/L、同时累及纵隔和淋巴结的患儿预后较差;减少大剂量氨甲蝶呤化疗次数能够降低重症感染率,但不影响预后。Objective To investigate the prognosis of childhood T-lymphoblastic lymphoma(T-LBL)treated with acute lymphoblastic leukemia(ALL)regimen and related influencing factors.Methods A retrospective analysis was performed for the prognostic characteristics of 29 children with T-LBL who were treated with ALL regimen(ALL-2009 or CCCG-ALL-2015 regimen)from May 2010 to May 2022.Results The 29 children with T-LBL had a 5-year overall survival(OS)rate of 84%±7%and an event-free survival(EFS)rate of 81%±8%.The children with B systemic symptoms(unexplained fever>38°C for more than 3 days;night sweats;weight loss>10%within 6 months)at initial diagnosis had a lower 5-year EFS rate compared to the children without B symptoms(P<0.05).The children with platelet count>400×10^(9)/L and involvement of both mediastinum and lymph nodes at initial diagnosis had lower 5-year OS rates(P<0.05).There were no significant differences in 5-year OS and EFS rates between the children treated with CCCG-ALL-2015 regimen and those treated with ALL-2009 regimen(P>0.05).Compared with the ALL-2009 regimen,the CCCG-ALL-2015 regimen reduced the frequency of high-dose methotrexate chemotherapy and the incidence rate of severe infections(P<0.05).Conclusions The ALL regimen is safe and effective in children with T-LBL.Children with B systemic symptoms,platelet count>400×10^(9)/L,and involvement of both mediastinum and lymph nodes at initial diagnosis tend to have a poor prognosis.Reduction in the frequency of high-dose methotrexate chemotherapy can reduce the incidence rate of severe infections,but it does not affect prognosis.

关 键 词：T淋巴母细胞淋巴瘤 治疗 预后 儿童 

分 类 号：R733.1[医药卫生—肿瘤]