儿童伯基特淋巴瘤/白血病35例临床特征及预后分析

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

目的:探讨儿童伯基特淋巴瘤/白血病(BL/B-AL)的临床特征及预后影响因素.方法:收集2011年3月到2017年9月本院共诊断35例BL初诊患儿临床资料,并进行分析总结.在35例中5例放弃治疗,1例未化疗即因多脏器功能衰竭死亡,29例接受中华医学会儿科学会儿科学分会血液学组制定的儿童和青少年成熟B淋巴非霍奇金淋巴瘤方案(CCCG-B-NHL)治疗至少2疗程化疗.结果:男31例,女4例,男∶女为7.75∶1,初诊中位年龄5(2.0-11)岁.常见的肿瘤浸润部位包括:腹腔(特别是回盲部)21例(60.00%),骨髓21例(60.00%),颌面部10例(28.57%),中枢神经系统6例(17.4%).根据St.Jude分期系统临床分期为:Ⅱ期6例(17.14%),Ⅲ期8例(22.86%),Ⅳ期(包括伯基特白血病)21例(60.00%);21例Ⅳ期患儿均有骨髓浸润,其中17例骨髓幼稚细胞数超过25%.疗效及预后分析显示,中位随访23.4(5.3-86.4)个月,5例复发,复发率17.24%(5/29),中位复发时间为5.7(3.9-7.2)个月;2年总体生存率(0S)为79.2%±7.6%,无进展生存率(PFS)为78.3%±7.9%.单因素分析显示,LDH>2N、Ⅳ期(骨髓浸润)、中枢神经系统浸润和2个疗程未达完全缓解(CR)组的2年0S及PFS显著低于LDH≤2N、Ⅱ+Ⅲ期、中枢神经系统无浸润和2疗程达CR组(P值分别为:0.015、0.015、0.019、0.000);Cox回归模型分析提示,2个疗程化疗后未达CR是儿童BL/B-AL独立的预后不良因素(HR0.034,95%CI:0.03-0.407).结论:儿童BL/B-AL多见于男性、学龄期,临床进展快,病情凶险,就诊时已多为晚期,骨髓及中枢神经系统浸润多见;高剂量、短疗程化疗效果显著,但如果2个疗程化疗后未达CR则预后不良.

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.