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Title: [Clinical Characteristics and Prognostic Factors of Children with Non-Hodgkin's Lymphoma of Different Pathological Subtypes].
Author: Zheng YZ, Chen ZS, Hua XL, Zheng H, LE SH, Li J, Hu JD.
Journal: Zhongguo Shi Yan Xue Ye Xue Za Zhi; 2020 Apr; 28(2):500-506. PubMed ID: 32319386.
Abstract:
OBJECTIVE: To investigate the clinical characteristics and prognostic factors of children with non-Hodgkin's lymphoma of different pathological subtypes. METHODS: Ninety-three patients with newly-diagnosed childhood NHL in Fujian Medical University Union Hospital from March 2011 to September 2017 were salected. The diagnosis of patients was performed according to the World Health Organization classification of tumors 2008 ys. The chemotherapy regimens were based on immune phenotype, pathological type and clinical stages. The 5-years event-free survival rate (EFS) were calculated and analyzed by Kaplan-Meier method, and difference of survival rate between groups were compared. The possible factors influencing 5-years EFS was analyzed using Cox proportional hazards model. RESULTS: Among the 93 patients, male to female ratio was 2.88:1, the median age at diagnosis was 6 (0.9 to 13) years old. According to pathological types, Burkitt's lymphoma was the most common, follow by ALK⁺ anaplastic large-cell lymphoma (ALCL) and lymphoblastic lymphoma (LBL). Clinically, the most common initial symptoms observed at diagnosis were swelling of superficial lymph node, and abdominal pain and abdominal mass in mature B-cell neoplasms, and the swelling of mediastinal lymph nodes in LBL, and hemophagocytic syndrome (HPS) in mature T-cell and natural killer cell NHL. Seventy-nine cases completed 2 courses of induction chemotherapy, and 64 cases (81.01%) reached complete remission (CR). In a median follow up for 32.5(1.0-88.5) months, ten patients (11.90%) relapsed, the median relapsed time was 5.7(3.4-15.7) months. 5-year EFS rate in 84 patients received standardized treatments were (77.1±4.9)%. As compared with lymphoblastic lymphoma and extranodal NK/T cell lymphoma, there was a trend towards better outcomes in B-LBL, and mature B-cell neoplasms and ALK⁺ ALCL showing 5-year EFS was (86.2±5.2)% and (93.8±6.1)% vs (53.3%±16.1)% and (28.6±17.1)%. Univariate analysis showed that B symptoms, LDH level, secondary HLH, immunophenotype, pathological subtypes, clinical stage and whether reached CR after induction chemotherapy significantly correlated with prognosis. Cox regression analysis showed that no CR after 2 courses was an independent unfavorable prognostic factor (HR0.001, 95%CI: 0.000-0.122). CONCLUSION: The clinical characteristics and prognosis of patients with NHL of different pathological types are different. Whether reached CR after induction chemotherapy is the imdependent risk factor affecting the prognosis. 题目: 儿童不同病理亚型非霍奇金淋巴瘤的临床特征及预后因素. 目的: 研究儿童不同病理亚型非霍奇金淋巴瘤（NHL）的临床特征及预后影响因素. 方法: 收集2011年3月到2017年9月期间福建医科大学附属协和医院收治的初发儿童NHL 93例，按照WHO-2008淋巴系恶性肿瘤分类标准诊断，根据不同免疫标记、病理类型及肿瘤分期选择化疗方案；采用Kaplan-Meier方法计算5年无事件生存率（EFS），用Cox比例风险模型对预后影响因素进行分析. 结果: 93例NHL患儿中，男女比例为2.88∶1，中位年龄6（0.9-13）岁，病理亚型伯基特淋巴瘤（BL）、ALK⁺间变大细胞淋巴瘤（ALCL）、淋巴母细胞淋巴瘤（LBL）、弥漫大B细胞淋巴瘤（DLBCL）和结外NK/T细胞淋巴瘤（ENKTL）分别为35、17、17、11和7例。在临床上，浅表淋巴结肿大为最常见的首诊表现，而腹腔瘤块（尤其是回盲部）伴LDH明显升高最常见于成熟B-NHL（B-NHL），纵膈肿物最常见于LBL，噬血细胞综合征（HPS）最常见于成熟T/NK细胞型NHL。79例完成2个疗程诱导化疗并评估，总体完全缓解（CR）率为81.01%；中位随访时间为32.5（1.0-88.5）个月，10例复发，复发率为11.90%，中位复发时间为5.9（3.4-15.7）个月；总体5年EFS为（77.1±4.9）%，T-LBL、B-LBL、成熟B淋巴细胞淋巴瘤、ALK⁺ALCL及ENKTL的5年EFS分别为（53.5±16.1）%、100%、（86.2±5.2）%、（87.5±8.3）%和（28.6±17.1）%。单因素分析显示，B症状、LDH水平、继发HLH、免疫分型、病理亚型、临床分期、诱导化疗是否CR等因素影响患儿的预后。多因素分析显示，诱导化疗2个疗程能否CR为独立的预后危险因素（HR0.001, 95%CI:0.000-0.122）. 结论: 不同病理亚型的儿童NHL临床特征及预后不同，诱导化疗后能否达CR是儿童NHL独立的预后因素.

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