Guo Jia, Zhu Yi-Ping, Gao Ju, Li Qiang, Jia Cang-Song, Zhou Chen-Yan, Guo Xia
Department of Pediatrics, West China Second Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
Department of Pediatrics, West China Second Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China. E-mail:
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2016 Feb;24(1):72-9. doi: 10.7534/j.issn.1009-2137.2016.01.014.
To explore the clinical and prognostic features as well as treatment response of childhood B-cell non-Hodgkin's lymphoma/acute lymphoblastic leukemia (B-NHL/B-ALL), so as to better modify the treatment for further improving the prognosis.
The clinical data of 43 patients with newly-diagnosed childhood B-NHL/B-ALL from July 2005 to December 2013 in West China Second Hospital of Sichuan University were retrospectively analyzed with particular focus on clinical presentations, laboratory findings and histology. Among them 26 patients received B-NHL-2010 protocol and 17 patients received LMB-89 protocol treatment. Kaplan-Meier method was used to compare the survival rates between groups, while multiple factor logistic regression was used to identify the prognostic factors.
(1) The median age at diagnosis was 7.58 (2.42-13.67) years. The male-to-female ratio was 2.9 : 1. No significant difference was found in the median age at diagnosis between male and female children with B-NHL/B-ALL (P = 0.837). (2) Burkitt's lymphoma was the most common (34/43, 79.07%), followed by diffuse large B cell lymphoma (4/43, 9.3%), ALL-L3 (3/43, 6.98%) and others (2/43, 4.65%) in decreasing frequency. (3) According to St. Jude staging classification, 4 patients (9.30%) were divided into stage I, 9 patients (20.93%) into stage II, 23 patients (53.49%) into stage III and 7 patients (16.28%) into stage IV; (4) Clinically, the common predilection sites were as following: ileocecus (11/43, 25.58%), nasopharynx (10/43, 23.26%), faciomaxillary (9/43, 20.93%), superficial lymphadenopathy (8/43, 18.60%), other sites such as mediastinum and bone marrow (5/43, 11.63%). (5) With a median follow up of 24 months (0.7-105 months), the 2-year overall survival (OS) rate and event-free survival (EFS) rate were 79.8% ± 6.5%% and 71.0% ± 7.2%, respectively. The 2-year OS and EFS rates in patients treated with B-NHL-2010 protocol were 79.1% ± 8.4% and 74.1% ± 8.4%, while those in patients treated with LMB-89 protocol were 87.5% ± 8.3% and 66.7% ± 12.4%, respectively, but there was no significant difference between them (P > 0.05). The 2-year EFS rate in patients with LDH > 2N and bone marrow infiltration were significantly lower than that of other groups (P < 0.05). (6) 8 patients (18.6%) relapsed. The median relapsed time was 6 months (2-9 months). 1 patient suffered progressive disease. Male, systemic symptom, elevated LDH, bone marrow and CNS infiltration and advanced stage (stage III and stage IV) were associated with relapse /progressive disease. Logistic regression analysis showed that LDH > 2N was an independent unfavorable prognostic factors (OR = 31.129, P = 0.02).
Outcome of B-NHL/B-ALL is greatly improved by current intensive and short-time chemotherapy regimen. The 2-year event-free survival (EFS) rate is 71.0% ± 7.2%. There is no significant difference in EFS rate between patients treated with B-NHL-2010 protocol and LMB89 protocol. The long-term survival rate in patient with advanced disease need to be further improved.
探讨儿童B细胞非霍奇金淋巴瘤/急性淋巴细胞白血病(B-NHL/B-ALL)的临床及预后特征以及治疗反应,以便更好地调整治疗方案以进一步改善预后。
回顾性分析2005年7月至2013年12月在四川大学华西第二医院新诊断的43例儿童B-NHL/B-ALL患者的临床资料,特别关注临床表现、实验室检查结果和组织学。其中26例患者接受B-NHL-2010方案治疗,17例患者接受LMB-89方案治疗。采用Kaplan-Meier法比较组间生存率,同时采用多因素logistic回归分析确定预后因素。
(1)诊断时的中位年龄为7.58(2.42 - 13.67)岁。男女比例为2.9 : 1。B-NHL/B-ALL的男童和女童诊断时中位年龄差异无统计学意义(P = 0.837)。(2)伯基特淋巴瘤最常见(34/43,79.07%),其次为弥漫性大B细胞淋巴瘤(4/43,9.3%)、ALL-L3(3/43,6.98%)和其他类型(2/43,4.65%),频率依次降低。(3)根据圣裘德分期分类,4例(9.30%)为Ⅰ期,9例(20.93%)为Ⅱ期,23例(53.49%)为Ⅲ期,7例(16.28%)为Ⅳ期;(4)临床上常见的好发部位如下:回盲部(11/43,25.58%)、鼻咽部(10/43,23.26%)、面颌部(9/43,20.93%)、浅表淋巴结(8/43,18.60%)、其他部位如纵隔和骨髓(5/43,11.63%)。(5)中位随访24个月(0.7 - 105个月),2年总生存率(OS)和无事件生存率(EFS)分别为79.8% ± 6.5%和71.0% ± 7.2%。接受B-NHL-2010方案治疗患者的2年OS和EFS率分别为79.1% ± 8.4%和74.1% ± 8.4%,而接受LMB-89方案治疗患者的分别为87.5% ± 8.3%和66.7% ± 12.4%,但两者差异无统计学意义(P > 0.05)。乳酸脱氢酶(LDH)> 2倍正常上限(2N)和骨髓浸润患者的2年EFS率显著低于其他组(P < 0.05)。(6)8例(18.6%)患者复发。中位复发时间为6个月(2 - 9个月)。有1例患者病情进展。男性、全身症状、LDH升高、骨髓和中枢神经系统浸润以及晚期(Ⅲ期和Ⅳ期)与复发/病情进展相关。logistic回归分析显示LDH > 2N是独立的不良预后因素(OR = 3I.129,P = 0.02)。
目前的强化短程化疗方案使B-NHL/B-ALL的预后有了很大改善。2年无事件生存率(EFS)为71.0% ± 7.2%。接受B-NHL-2010方案和LMB89方案治疗患者的EFS率差异无统计学意义。晚期疾病患者的长期生存率有待进一步提高。