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目的:分析我中心2007年至2013年确诊的34例Burkitt淋巴瘤(BL)的病理、临床特征及12例临床特点和预后关系。方法:回顾分析2007年-2013年我中心确诊的34例BL(占淋巴瘤总数1.43%,B细胞淋巴瘤2.03%)的病理资料及12例接受治疗的患者临床资料,治疗方法为[hyper CVAD(A+B)±R(利妥昔单抗)]±自体造血干细胞移植(allo-HSCT),观察短期疗效[完全缓解(CR)和部分缓解(PR)]和长期疗效[包括1年和2年总生存率(OS)及无进展生存率(PFS)],分析预后影响因素。结果:34例患者中男∶女=10∶7,中位年龄23.5岁(3~74岁),0~14岁29.4%(10/34),15~22岁23.5%(8/34),大于22岁47.1%(16/34)。全身各脏器均可发病,主要以浅表淋巴结、脑部及鼻咽部为主。Ki-67指数均大于95%以上;c-myc阳性77%(10/13)。其中12例在我中心接受治疗,Ⅱ期1例(8.4%),Ⅲ-Ⅳ期11例(91.7%)。诱导治疗:11例(91.7%)采用hyper CVAD(A+B)方案化疗,3疗程后2例达PR,9例(82%)达CR。9例达CR的患者中有1例采用CHOPE方案巩固化疗,1年内复发;5例采用hyper CVAD(A+B)巩固化疗6疗程,1例1年后复发,2年OS及PFS分别为100%、80%。3例采用hyper CVAD(A+B)+利妥昔单抗(R)方案巩固化疗6疗程,2年OS及PFS均为100%,PFS较未用R组高。3例在巩固、维持治疗后序贯allo-HSCT,随访至2年无复发,病情稳定,OS及PFS均为100%。随访2年无复发8例,年龄小于15岁占50%(4/8),15~22岁占37.5%(3/8),大于22岁占12.5%(1/8)。结论:BL主要发生在儿童和青少年,男性高于女性,可累及全身各个器官,诊断时临床分期常较高,需要高强度、高频率、足疗程、规律化疗,尤其是儿童及青少年患者。利妥昔单抗和大剂量化疗药物协同使用,序贯自体造血干细胞移植巩固治疗能提高OS率与PFS,但仍需要临床大样本研究。
Objective: To analyze the pathology and clinical features of 34 cases of Burkitt lymphoma (BL) diagnosed in our center from 2007 to 2013 and the relationship between the clinical features and prognosis of 12 cases. Methods: The pathological data of 34 cases of BL (1.43% of total lymphoma and 2.03% of B-cell lymphoma) diagnosed in our center from 2007 to 2013 were retrospectively analyzed. The clinical data of 12 patients treated by hyper CVAD (A + B) ± R (Rituximab)] ± autologous hematopoietic stem cell transplantation (allo-HSCT) to observe the short-term efficacy [complete remission (CR) and partial remission (PR) 2-year overall survival (OS) and progression-free survival (PFS)], analysis of prognostic factors. Results: Among the 34 patients, the male and female ratio was 10:7. The median age was 23.5 years (range, 3 to 74 years), 0 to 14 years old was 29.4% (10/34), 15 to 22 years old was 23.5% (8/34) 47.1% (16/34) older than 22 years. The body can be the incidence of various organs, mainly superficial lymph nodes, brain and nasopharyngeal dominated. Ki-67 index was greater than 95%; c-myc positive 77% (10/13). Among them, 12 cases were treated in our center, 1 case (8.4%) in stage Ⅱ and 11 cases (91.7%) in stage Ⅲ-Ⅳ. Induction therapy: Eleven patients (91.7%) were treated with hyper CVAD (A + B) regimen, 2 patients achieved PR after 3 cycles, and 9 patients (82%) achieved CR. One of 9 CR patients was treated with CHOPE regimen and relapsed within 1 year. Five patients were treated with hyper CVAD (A + B) for 6 courses of chemotherapy, one patient relapsed one year later. The 2-year OS and PFS were 100 %, 80%. 3 cases of hyper CVAD (A + B) + rituximab (R) regimen consolidation chemotherapy 6 courses, 2-year OS and PFS were 100%, PFS than the group without R high. Three cases were treated with allo-HSCT after consolidation and maintenance treatment. No recurrence was observed after 2 years of follow-up, and the disease was stable. The OS and PFS were 100%. Eight patients were followed up for 2 years without recurrence, 50% (4/8) younger than 15 years old, 37.5% (3/8) aged 15-22 and 12.5% (1/8) older than 22 years old. Conclusion: BL mainly occurs in children and adolescents. Men are higher than women and can affect various organs of the whole body. The clinical staging is often high and requires high intensity, high frequency, full course of treatment and regular chemotherapy, especially for children and adolescents. Rituximab and high-dose chemotherapy drugs synergistic use of sequential autologous stem cell transplantation consolidation therapy can improve the OS rate and PFS, but still need clinical large sample studies.