Burkitt淋巴瘤临床分析

Clinical analysis of the Burkitt lymphoma

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,clinical features of 34 cases of Burkitt lymphoma（ BL）,confirmed in our center from 2007 to 2013,and the relationship between the treatment and curative effect in 12 cases. Methods：To retrospectively analyze the pathological data of 34 cases of BL diagnosed in our center 2007- 2013（ accounting for1. 43% of the total number of lymphoma in our hospital,accounting for 2. 03% of B cell lymphoma） and the clinical data of 12 patients treated in our center. Chemotherapy [hyper CVAD（ A ＋ B） ± R（ rituximab） ]± autologous hematopoietic stem cell transplantation,observe the short- term remission rate[comoplete remission（ CR） and partial remission（ PR） ] and the long- term efficacy including 1 year and 2 years overall survival rate（ OS）,progression free survival（ PFS）. Results： Male∶ Female = 10∶ 7 of 34 diagnosed patients,the median age was 23. 5 years（ 3 - 74 years）,0 - 14 29. 4%（ 10 /34）,15 - 22 23. 5%（ 8 /34）,more than 22 47. 1%（ 16 /34）. It involves in various organs of the body（ mainly in lymph nodes,brain and nasopharynx）; Ki- 67 index were greater than 95%. Among them,12 cases treated in our center,1 case（ 8. 4%） in phase II,4 cases（ 33. 3%） in stage III,seven cases（ 58. 3%） in stage IV.Receiving treatment in 12 patients,1 case with bone marrow infiltration,after 1 course treatment of the VDCP chemotherapy,disease progression,death. 11 cases（ 91. 7%） were take 3 course of the treatment of hyper CVAD（ A ＋ B）chemotherapy,2 cases partial remission（ PR）,9 cases complete remission（ CR）,the complete remission rate was82%. In 9 cases of CR patients,1 case treated with CHOPE regimen chemotherapy,recurrence within 1 year,5 cases take hyper CVAD（ A ＋ B） to consolidate the 6 course of chemotherapy,1 case of recurrence after 1 year,the 2 year overall survival rate（ OS） and progression free survival（ PFS） were 100% and 80%. 3 cases were taked hyper CVAD（ A ＋ B） ＋ R regimen to consolidate the 6 course of chemotherapy,followed up for 2 years without recurrence,OS and PFS was 100% in 2 years,PFS was high compared with no R supplying. 3 patients were taked the treatment of autologous hematopoietic stem cell transplantation after 1 year of the consolidation and maintenance treatment,followed up for 2 years without recurrence,stable condition,OS and PFS were 100%. Among the patients（ 8 /11） with no recurrence and stable disease during 2 year follow- up,less than 15 years of age accounted for 50%（ 4 /8）,15 - 22 years old accounted for 37. 5%（ 3 /8）,more than 22 years old accounted for 12. 5%（ 1 /8）. Conclusion： Burkitt＇s lymphoma mainly occurs in children and adolescents,male is more than female,and it involves in various organs of the body. The diagnosis of clinical stage is often higher,chemotherapy with high strength,high frequency and regular,can improve the prognosis and raise the complete remission rate and total survival rate,especially to children and adolescents.Combined the consolidation of rituximab with autologous stem cell transplantation therapy can improve the rate of OS and PFS.