Burkitt淋巴瘤患者的临床特点及影响预后的因素分析  

Clinical Characteristics and Prognosis Affecting-Factors of the Patients with Burkitt Lymphoma

作　　者：杨萍[1] 董菲[1] 赵伟[1] 田磊[1] 万伟[1] 李其辉[1] 高锦洁[1] 克晓燕[1] 王继军[1] 景红梅[1] YANG Ping;DONG Fei;ZHAO Wei;TIAN Lei;WAN Wei;LI Qi-Hui;GAO Jin-Jie;KE Xiao-Yan;WANG Ji-Jun;JING Hong-Mei(Department of Hematology,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学第三医院血液科,北京100191

出　　处：《中国实验血液学杂志》2021年第5期1498-1503,共6页Journal of Experimental Hematology

摘　　要：目的:探讨Burkitt淋巴瘤(BL)患者的临床特点并分析其疗效。方法:收集并回顾性分析27例BL患者的临床资料,对患者临床特征及实验室指标进行分析,同时进行生存和预后影响因素分析。结果:本研究患者以成人为主,中位发病年龄为30(15-83)岁,男性∶女性为3.5∶1,所有患者无EB病毒感染,92.6%患者存在结外器官受累,其中40.7%的患者起病时为白血病表现,Ⅲ-Ⅳ期占85.2%,IPI评分高危/高中危组占74.1%;分子生物学方面,5例患者进行了二代测序,均具有MYC基因突变,最常见突变为CCND3、ID3、TP53突变。治疗总有效率为85.2%,完全缓解率为63.0%,部分缓解率为22.2%,5年无进展生存率和5年总生存率均为76.6%,高剂量甲氨蝶呤治疗组疗效优于未使用高剂量甲氨蝶呤治疗组,其中LMB89方案治疗组完全缓解率为78.6%,总有效率为100%,5年生存率为92.9%,显著优于其他治疗方案组;无法耐受高剂量化疗的患者进行自体造血干细胞移植作为巩固治疗,具有生存获益。单因素分析显示,ECOG评分、血清乳酸脱氢酶大于500 U/L、白细胞水平、是否存在中枢系统受累、近期疗效及是否应用R-LMB89方案为影响患者预后的因素。结论:成人BL具有高度侵袭性,高剂量化疗LMB89方案在本研究中显示出较好的疗效和生存率,对于无法耐受高剂量甲氨蝶呤患者,一线自体造血干细胞移植作为巩固治疗为患者可供选择的治疗方案。Objective:To investigate the clinical characteristics and treatment outcome of patients with Burkitt lymphoma.Methods:The clinical data of 27 patients with Burkitt Lymphoma were collected and retrospectively analyzed,the clinical characteristics,laboratory data,survival and the factors affecting the prognosis were also analyzed.Results:Among the 27 patients(mainly for adults),the median age was 30(15-83)years old,the ratio of male and female was 3.5:1.There was no EB virus infection in all the patients,92.6%of the patients showed extranodal organs involvement,40.7%of them were leukemic stage,85.2%patients belonged toⅢandⅣstage,74.1%patients belonged to high/high-middle risk according to IPI index.In the terms of molecular biology,five patients were treated with nextgeneration sequencing test,and the MYC gene mutations were detected out in alt the patients,and the most common mutations were CCND3,ID3 and TP53.The overall response rate(ORR)for all the patients was 85.2%,the complete response(CR)rate was 63.0%,and the partial response rate was 22.2%,the 5-year progression-free survival rate and overall survival rate of the patients was 76.6%and 76.6%,respectively,which showed that the efficacy of the patients in high-dose methotrexate treatment group was higher than that in the non-high high-dose methotrexate treatment group.For the patients treated with LMB89 chemotherapy,the CR was 78.6%,ORR was 100%,the 5-year survival rate was92.9%,which was superior to the patients treated with other regimens.Auto-hematopoietic stem cell transplantation as consolidation treatment could improve the prognosis for those patients who could not tolerate high-dose chemotherapy.Univariate analysis showed that ECOG score,the level of LDH>500 U/L,WBC level,CNS involvement,short-term effect and LMB89 regimen were the risk factors affecting the prognosis of the patients.Conclusion:The adult Burkitt lymphoma are highly aggressive.For the patients in high-dose methotrexate treatment group,especially LMB89 regimen can improve the surviva

关 键 词：BURKITT淋巴瘤 成人 临床特点 治疗 预后 

分 类 号：R733.4[医药卫生—肿瘤]