Clinical Analysis of 13 Cases of Burkitt Lymphoma and Burkitt-like Lymphoma

目的:分析伯基特淋巴瘤(BL)和伯基特样淋巴瘤(BLL)的临床特点,总结疗效,探讨可能的最佳方案和治疗相关合并症.方法:回顾性分析北京肿瘤医院1996年8月至2008年10月收治的13例经病理确诊为伯基特淋巴瘤和伯基特样淋巴瘤患者的临床资料.所有患者均接受化疗为主的治疗方案,评价疗效和不良反应.结果:13例患者中,男12例,女1例;发病年龄11～62岁,中位年龄15岁;Ⅰ期3例,Ⅱ期2例,Ⅲ期2例,Ⅳ期6例,其中晚期(Ⅲ、Ⅳ期)病例占61.5%,初治时发生骨髓侵犯2例(15.4%),中枢神经系统侵犯4例(30.8%);常见的侵犯部位为浅表淋巴结(61.5%)、腹腔脏器(53.8%)和腹腔及腹膜后淋巴结(38.5%);有B症状7例(53.8%);8/10例(80.0%)血清乳酸脱氢酶(LDH)水平升高,1/10例血清尿酸升高;病理示BL 11例,BLL2例.11例患者获得完全缓解或未经证实的完全缓解,1例部分缓解,总有效率为92.3%.中位随访时间8个月(5～35个月),至随访截止6例患者死亡,1例失访.1年总生存率(OS)、无进展生存率(PFS)和无瘤生存率(DFS)分别为56.98%、32.31%和39.77%.化疗中Ⅲ～Ⅳ度骨髓抑制发生率为69.2%,1例患者出现肿瘤溶解综合征和Ⅳ度全消化道黏膜炎.结论:推荐高强度短疗程化疗方案作为BL和BLL的一线治疗,应积极预防处理化疗不良反应.Objective:To summarize the clinical characteristics of Burkitt lymphoma(BL)and Burkitt-like lymphoma(BLL)and the effect of treatment on 13 cases,and to explore the treatment-related complications and optimal treatment.Methods:Clinical data of 13 BL and BLL patients treated between August 1996 and October 2008 in our hospital were retrospectively analyzed.All of these patients received chemotherapy as the first-line treatment.The efficacy and adverse reactions were evaluated.Results:Of the 13 patients,there were 12 males and 1 female,with a median age of 15 years(ranging from 11 to 62).There were 3 stage Ⅰ cases,2 stage Ⅱ cases,2 stage Ⅲ cases,and 6 stage Ⅳ cases.The advanced stage(stage Ⅲ and Ⅳ)patients accounted for 61.5%(8 cases).CNS was involved in 4 cases and bone marrow was involved in 2 cases at diagnosis.The commonly involved sites included superficial lymph nodes(61.5%),abdominal organs(53.8%),and celiac and retro-pentoneal lymph nodes(38.5%).B symptoms were observed in 7 patients(53.8%).Serum lactate dehydrogenase level was elevated in 8 of 10 cases,while serum udc acid level was elevated in 1 of 10 cases.Eleven patients were diagnosed as BL and 2 patients were diagnosed as BLL.Of the 13 patients,11(84.6%) achieved complete remission(CR)or CR/unconfirmed(CRu),and 1 patient(7.7%) got partial remission(PR).Dudng the follow-up of 8 months(ranging from 5 to 35),6 patients were still alive.The 1-year overall survival,progression-free survival and disease-free survival were 56.98%,32.31% and 39.77%,respectively.Nine patients(69.2%)developed grade Ⅲ or Ⅳ myelosuppression.Conclusion:Intensive short-course chemotherapy is the optimal first-line treatment for BL and BLL.中文核心期刊要目总览(PKU)中国科技核心期刊(ISTIC)中国科学引文数据库(CSCD)015-83