首都医科大学学报 ›› 2016, Vol. 37 ›› Issue (2): 141-147.doi: 10.3969/j.issn.1006-7795.2016.02.007

• 儿科新进展 • 上一篇    下一篇

儿童伯基特白血病26例临床研究

段彦龙, 金玲, 杨菁, 黄爽, 张梦, 赖瑞, 周春菊, 张永红   

  1. 首都医科大学附属北京儿童医院血液肿瘤中心 儿童血液病与肿瘤分子分型北京市重点实验室儿科学国家重点学科, 北京 100045
  • 收稿日期:2016-03-01 出版日期:2016-04-21 发布日期:2016-04-14
  • 通讯作者: 张永红 E-mail:yhzhang58@hotmail.com
  • 基金资助:
    天津医科大学科学基金(2009KY19)

Clinical characteristics of 26 children with Burkitt's leukemia and outcome of treatment with revised LMB 89 protocol

Duan Yanlong, Jin Ling, Yang Jing, Huang Shuang, Zhang Meng, Lai Rui, Zhou Chunju, Zhang Yonghong   

  1. Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Ministry of Education, Beijing 100045, China
  • Received:2016-03-01 Online:2016-04-21 Published:2016-04-14
  • Supported by:
    This study was supported by Tianjin Medical University Science Foundation(2009KY19).

摘要: 目的 认识儿童伯基特白血病的临床特点,总结改良LMB89方案治疗伯基特白血病的疗效,探讨疾病相关严重合并症的防治。方法 研究2010年1月至2015年4月于首都医科大学附属北京儿童医院初治的伯基特白血病患儿;治疗前全部行骨髓形态学(骨髓肿瘤细胞浸润>25%)、白血病免疫分型及骨髓活检病理及C-MYC基因检查确诊,全部采用高剂量、短疗程的改良LMB89方案的C组治疗。结果 规范治疗伯基特白血病患儿26例,其中男24例,女2例;平均年龄7.8岁,临床表现中2例以急性白血病起病,无其他淋巴结等部位肿瘤浸润, 23例患儿伴有腹部瘤块;16例患儿合并颌面部包块;7例患儿有睾丸受累,6例患儿伴中枢神经系统白血病(central nervous system leukemia, CNSL);初诊时全部存在严重高乳酸脱氢酶(lactate dehydrogenase, LDH)血症,24例患儿存在高尿酸血症,18例患儿化学药物治疗(以下简称化疗)初期伴有严重肿瘤溶解综合征。所有患儿化疗期间都合并IV度骨髓抑制,其中20例联合利妥昔单抗治疗。患儿平均随访时间35个月,2年整体生存率(overall survival, OS)及无事件生存率(event free survival,EFS)均为76.9%。危险因素分析提示初诊时乳酸脱氢酶(lactate dehydrogenase, LDH)大于10倍以及治疗早期合并严重感染败血症为预后不良因素。2例死于早期严重骨髓抑制期并发败血症,4例患儿化疗7个月内早期进展或复发死亡。结论 儿童时期伯基特白血病不同于普通急性淋巴细胞白血病,是成熟B细胞淋巴瘤的晚期状态,相对多见于学龄期,临床进展快、过程凶险,易合并肿瘤溶解综合征。高剂量、短疗程的改良LMB89化疗方案治疗效果显著,由于肿瘤特性及瘤负荷巨大导致的早期持续的严重骨髓抑制并发感染,以及治疗后期的早期复发是影响预后的主要因素。

关键词: 伯基特淋巴瘤, 白血病, 儿童, 治疗, 临床研究性

Abstract: Objective To summarize the clinical features and to evaluate outcomes and to assess therapeutic effects in 26 children and adolescents with Burkitt's leukemia treated with revised LMB 89 protocol in China.Methods From January 2010 to April 2015, 26 hospitalized children and adolescents with Burkitt's leukemia were enrolled into this study. Staging was based on clinical evaluation and was defined by the St. Jude staging system. All patients were confirmed to have Burkitt's leukemia and mature B blast > 25% in marrow that diagnosed by MICM system and biopsy of marrow. The 26 patients were treated with C group of the revised LMB 89 protocol.All analyses were performed by the statistical program SPSS.Results Of the 26 Burkitt's leukemia patients, 24 were male and 2 were female.The median age was 7.8 years (range from 3 years to 13 years) at the time of diagnosis.In terms of clinical presentation, 88% had abdominal bulky lymphoma involvement; in 62% of the patients maxillofacial region was involved, 27% had testis involvement, 69% had severe tumor lysis syndrome. All patients were followed up. The overall survival (OS) and the 2-year event-free survival was 76.9% respectively at a median follow-up of 35 months.All patients suffered from IV degree marrow suppression and sever infection. Two patients died from sepsis in the early 2 course of chemotherapy because of severe marrow suppression, and no child died from TLS. Three patients had early relapse after treatment was finished.Conclusion Childhood Burkitt's leukemia in our study was more frequently seen in male school aged children.Revised LMB 89 protocol used for Burkitt's leukemia is effective and well tolerated if the early TLS could be controlled.The overall prognosis is worse than that of other group Burkitt's lymphoma because of early severe persistent marrow suppression complicated with sepsis.The early relapse is still a challenge.

Key words: Burkitt's lymphoma, leukemia, child, therapies, investigational

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