13例伯基特和伯基特样淋巴瘤的临床特点分析

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BL11例，BLL 2 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。 

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

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.