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[35例伯基特淋巴瘤/白血病患儿的临床特征与预后]

[Clinical Features and Prognosis of 35 Children with Burkitt Lymphoma/Leukemia].

作者信息

Zheng Yong-Zhi, LE Shao-Hua, Zheng Hao, Hua Xue-Ling, Chen Zai-Sheng, Zheng Ling, Chen Cai, Li Mei, Cai Chun-Xia, Yang Jing-Hui, Chen Yi-Qiao, Gao Qin-Li, Chen Ying-Ying, Li Jian, Hu Jian-Da

机构信息

Department of Pediatric Hematology, Fujian Provincial Institute of Hematology, Fujian Provincial Key Laboratory of Hematology, Fujian Medical University Union Hospital, Fuzhou 350001, Fujian Provincial, China.

Department of Pediatric Hematology, Fujian Provincial Institute of Hematology, Fujian Provincial Key Laboratory of Hematology, Fujian Medical University Union Hospital, Fuzhou 350001, Fujian Provincial, China,E-mail:

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2019 Aug;27(4):1123-1130. doi: 10.19746/j.cnki.issn.1009-2137.2019.04.021.

Abstract

OBJECTIVE

To investigate the clinical features and prognostic factors of childhood Burkitt Lymphoma/leukemia.

METHODS

The clinical data of 35 patients with newly-diagnosed childhood Burkitt lymphoma/leukemia from March 2011 to September 2017 in Fujian Medical University Union Hospital were retrospectively analyzed and summarized. Among 35 patients, 5 gave up treatment and one patient died of multiple organ failure before treatment, and 29 patients received CCCG-BNHL-2010 protocol chemotherapy.

RESULTS

The 35 cases of BL/L includsd 31 males and 4 females (M∶F=7.75∶1) with the median age of 5(2.0-11) years. Clinically, the common infiltration sites were as follows: abdominal organs (especially ileocecus, 21/35, 60%), bone marrow (21/35, 60%), faciomaxillary (10/35, 28.57%), and central nervous system (8/35, 22.85%). According to St. Jude staging system, 6 patients were grouped into stage Ⅱ, and 8 into stage Ⅲ and 21 into stage Ⅳ, among which the bone marrow blasts of 17 patients were more than 25%. The analysis of therapeutic efficacy and prognosis showed that in median follow up of 23.4 (5.3-86.4) months, 5 patients relapsed (5/29, 17.24%), the median relapsed time was 5.7 (3.9-7.2) months; tow-year overall survival (OS) rate and progression-free survival (PFS) rate was 79.2%±7.6% and 78.3%±7.9%, respectively. Univariate analysis showed that the 2-year OS and PFS in patients with LDH>2N, stage Ⅳ (bone marrow infiltration), central nervous system infiltration and no-CR after 2 courses of treatnent all were significantly lower than those in patients with LDH≤2N, stageⅡ-Ⅲ, without central nervous system infiltration as well as CR after 2 course of treatment (P values were 0.015, 0.015, 0.019 and 0.000, respectively). Cox regression analysis showed that no-CR after 2 course was an independent unfavorable prognostic factor (HR 0.34, 95%CI: 0.03-0.407).

CONCLUSION

The childhood Buruitts lymphoma/leukemia is more freguently seen in males and school-age children, Advanced stage, bone marrow and contral nervous system infitration are common at the first visit to doctor, moreover the Burkitt's lymphoma/leykemia present repid progression and dangerous feature. The current intensive chemotherapy (high dose of drugs and short course) possess the significant therapeutic efficacy for this disease, but the patients should have very poor prognosis if they can not achieve CR after 2 course of chemotherapy.

摘要

目的

探讨儿童伯基特淋巴瘤/白血病的临床特征及预后因素。

方法

回顾性分析并总结2011年3月至2017年9月在福建医科大学附属协和医院新诊断的35例儿童伯基特淋巴瘤/白血病患者的临床资料。35例患者中,5例放弃治疗,1例在治疗前死于多器官功能衰竭,29例患者接受CCCG-BNHL-2010方案化疗。

结果

35例BL/L患者中,男31例,女4例(男∶女=7.75∶1),中位年龄5(2.0 - 11)岁。临床上,常见浸润部位如下:腹部器官(尤其是回盲部,21/35,60%)、骨髓(21/35,60%)、面颌部(10/35,28.57%)和中枢神经系统(8/35,22.85%)。根据圣裘德分期系统,6例患者为Ⅱ期,8例为Ⅲ期,21例为Ⅳ期,其中17例患者骨髓原始细胞超过25%。治疗疗效及预后分析显示,中位随访23.4(5.3 - 86.4)个月,5例患者复发(复发率5/29,17.24%),中位复发时间为5.7(3.9 - 7.2)个月;两年总生存率(OS)和无进展生存率(PFS)分别为79.2%±7.6%和78.3%±7.9%。单因素分析显示,乳酸脱氢酶(LDH)>2倍正常值上限、Ⅳ期(骨髓浸润)、中枢神经系统浸润以及2疗程治疗后未达完全缓解(CR)的患者,其2年OS和PFS均显著低于LDH≤2倍正常值上限、Ⅱ - Ⅲ期、无中枢神经系统浸润以及2疗程治疗后达CR的患者(P值分别为0.015、0.015、0.019和0.000)。Cox回归分析显示,2疗程治疗后未达CR是独立的不良预后因素(风险比HR 0.34,95%可信区间CI:0.03 - 0.407)。

结论

儿童伯基特淋巴瘤/白血病在男性及学龄儿童中更为常见,就诊时晚期、骨髓及中枢神经系统浸润常见,且伯基特淋巴瘤/白血病进展迅速、危险性高。目前的强化化疗(高剂量药物及短疗程)对该病具有显著疗效,但2疗程化疗后未达CR的患者预后极差。

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