儿童T淋巴母细胞淋巴瘤35例临床分析

Clinical Analysis of 35 Children with T Lymphoblastic Lymphoma

目的探讨儿童T淋巴母细胞淋巴瘤(T-LBL)的临床特征及预后。方法回顾性分析2014年8月至2022年2月郑州大学第一附属医院收治的35例T-LBL患儿的临床资料,依据性别、有无发热、盗汗、体重减轻的全身症状(B症状)、有无胸腔积液、乳酸脱氢酶(LDH)水平、临床分期、是否复发进行分组,采用Kaplan-Meier法计算无事件生存(EFS)率和总生存(OS)率,应用log-rank检验比较不同组间生存差异。结果35例T-LBL患儿中男24例(68.6%),女11例(31.4%),诊断时中位年龄9(7,11)岁,骨髓受累7例(20.0%),中枢神经系统浸润2例(5.7%),Ⅱ、Ⅲ、Ⅳ期患儿分别为1例(2.9%)、25例(71.4%)、9例(25.7%)。TdT、CD99、CD3、CD7阳性率分别为91.4%、100.0%、94.2%、100.0%,不同程度表达CD20、CD34、CD10、CD21、CD30。35例患儿3年OS率、EFS率分别为85.6%和74.4%。单因素分析显示,性别、LDH水平、复发是3年OS率的影响因素(P<0.05)。7例复发患儿的复发中位时间为10.9(5.0,28.0)个月,复发患儿3年OS率为38.1%。结论T-LBL是一种侵袭性高、预后差的恶性肿瘤,女性患儿、高LDH、复发可能提示患儿预后不良。

Objective To investigate the clinical features and prognosis of children with T-cell lymphoblastic lymphoma(T-LBL).Methods Retrospective analysis was performed for the medical data of 35 children with T-LBL admitted to the First Affiliated Hospital of Zhengzhou University from August 2014 to February 2022,which was grouped according to gender,age,systemic symptoms included fever,night sweats,and weight loss(B symptoms),pleural effusion,lactate dehydrogenase(LDH),clinical stage and recurrence.Overall survival(OS)and event free survival(EFS)rates were calculated by the Kaplan-Meier method.Survival differences between the groups was performed with log-rank test.Results Among the 35 patients included in the current study,24 patients(68.6%)were males and 11 patients(31.4%)were females.The median age of onset was 9(7,11)years,7 patients(20.0%)exhibited bone marrow infiltration and 2 patient(5.7%)showed central nervous system involvement.There were 1 patient with stage Ⅱ T-LBL(2.9%),25 patients with stage Ⅲ T-LBL(71.4%),and 9 patients with stage Ⅳ T-LBL(25.7%).Positive rate of terminal deoxynucleotidyl transferase(TdT),CD99,CD3 and CD7 were 91.4%,100.0%,94.2%and 100.0%,respectively,and different degrees of CD20,CD34,CD10,CD21 and CD30 expression were demonstrated.The 3-year OS rate and EFS rate were 85.6% and 74.4%,respectively.Univariate analysis showed that gender,LDH level and recurrence were the influencing factors of 3-year OS rate(P<0.05).For 7 children with recurrence,the median time of recurrence was 10.9(5.0,28.0)months and the 3-year OS rate was 38.1%.Conclusion T-LBL is a malignant tumor with high aggressiveness and poor prognosis.Female patients,high LDH and recurrence may indicate poor prognosis in children with T-LBL.