成人散发性伯基特淋巴瘤/白血病临床分析

Clinical analysis of adults patients with sporadic Burkitt lymphoma/leukemia

目的探讨成人散发性伯基特淋巴瘤/白血病(BL)的临床特征、治疗方案,为临床治疗提供依据。方法回顾性分析2013年6月至2019年6月郑州大学第一附属医院收治的31例成人BL患者的临床资料。结果31例BL患者中,男18例,女13例,中位发病年龄30(16~65)岁;常见的侵犯部位为腹腔/盆腔器官20例(64.5%),颈部12例(38.7%),骨髓8例(25.8%);EB病毒编码的小RNA阳性率为5/17(29.4%);MYC基因易位阳性率为13/15(86.7%),2例MYC基因无易位患者中1例伴11q异常。患者平均生存时间(17.51±3.08)个月,1 a总体生存率为60.1%。单因素分析显示,年龄≥50岁、治疗未达完全缓解(CR)、β2微球蛋白升高可以降低患者的1 a总体生存率(P<0.05)。结论成人散发性BL易侵犯结外器官,患者生存期短,年龄≥50岁、治疗效果未达CR、血清β2微球蛋白水平升高是患者的不良预后因素。

Objective To provide basis for clinical treatment of the patients with adult sporadic Burkitt lymphoma/leukemia(BL)by investigate the clinical characteristics and therapeutic regimens.Methods The clinical data of 31 patients who were diagnosed BL in the First Affiliated Hospital of Zhengzhou University from June 2013 to June 2019 was statistically analyzed retrospectively.Results Among the 31 BL patients,there were 18 males and 13 females,with the median age of onset being 30(range 16-65)years old.The common affected organs included abdominal/pelvic organs in 20 cases(64.5%),12 cases of neck(38.7%),8 cases of bone marrow(25.8%).The rate of overexpression of Epstein barr virus encoded RNA(EBER)was 5/17(29.4%)and MYC translocation was 13/15(86.7%).Among the 2 patients without translocation of MYC gene,1 patient with 11q abnormal BL was found.The average survival time of the patients was(17.51±3.08)months,the 1-year overall survival rate was 60.1%.Univariate analysis results showed that patients with≥50 years of age,the treatment effect did not achieved complete remission(CR)and elevatedβ2 microglobulin could reduce the 1-year overall survival rate of patients with BL(P<0.05).Conclusion The patients with adult sporadic BL is prone to invade extraterritoral organs,and the survival time of the patients is short.Age≥50 years old,treatment effect not achieve CR and serum levels ofβ2 microglobulin increasing were the poor prognostic factors of the patients.