13例伯基特和伯基特样淋巴瘤的临床特点分析

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月收浩的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,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 Oc tober 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 Ⅳcases. 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 diag nosis. The commonly involved sites included superficial lymph nodes （61.5%）, abdominal organs （53.8%）, and celiac and retro-peritoneal 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 uric 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 CP-Junconfirmed （CRu）, and 1 patient （7.7%） got partial remission （PR）. During 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.