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（aute lymphoblastic leukemia, 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3 d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>400×109/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>400×109/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。
Abstract
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 >400×109/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 >400×109/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
Key words
T-lymphoblastic lymphoma /
Treatment /
Prognosis /
Child
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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）。
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