摘要
目的:分析血管免疫母细胞性T细胞淋巴瘤(AITL)的临床特点及采用蒽环类药物为基础化疗的疗效和远期生存,并分析全血EBV-DNA及病理组织EBER的表达情况及其对预后的影响。方法:回顾性分析我院2015年1月—2020年3月收治的34例初治AITL患者的临床资料。结果:本组患者中位发病年龄为61(33~78)岁,男女比例为0.62︰1.00,Ⅲ/Ⅳ期患者比例达97.1%,易合并免疫现象。一线方案采用CHOP/CHOEP方案联合或不联合西达本胺治疗,总体完全缓解率为29.4%,总有效率为61.8%。中位随访时间为24(1~59)个月。整组患者中位总生存(OS)为57个月,1年及2年OS率分别为64.6%和56.0%。中位无进展生存(PFS)为11个月,1年及2年PFS率分别为41.3%和27.6%。西达本胺+CHOP/CHOEP组的OS及PFS曲线均呈现优于CHOP/CHOEP组的趋势,但差异无统计学意义(P=0.135和P=0.135)。25例(73.5%)患者存在全血EBV-DNA或病理标本EBER检测至少一项阳性。治疗前全血EBV-DNA阳性AITL患者的OS及PFS相对于EBV-DNA阴性者较差(P=0.034和P=0.057),但病理组织EBER的状态对生存并无显著影响。结论:AITL好发于老年人,起病多为疾病晚期,常合并自身免疫现象。EBV参与了多数AITL患者的发病,全血EBV-DNA阳性与AITL的不良预后相关,EBER状态对生存无显著影响。AITL患者仅应用CHOP/CHOEP方案化疗所获得的疗效并不令人满意,西达本胺联合化疗有望提高化疗的有效率及改善AITL患者的远期生存。
Objective: To evaluate the clinical characteristics, the efficacy of anthracycline-based regimen and long-term survival of angioimmunoblastic T cell lymphoma(AITL). The prognostic value of whole blood EBV-DNA and pathological tissue EBER status was also analyzed. Methods: Between January 2015 and March 2020, clinical data of 34 newly diagnosed AITL patients were retrospectively reviewed. Results: The median age at diagnosis was 61(range, 33-78) years, with male to female ratio of 0.62︰1.00. The 97.1% of patients were in stage Ⅲ/Ⅳ. Autoimmune phenomenon was common. The first-line treatments were CHOP/CHOEP regimen with or without chidamide. The complete remission rate was 29.4% and overall response rate was 61.8%. The median follow-up time was 24(range, 1-59) months. The median overall survival(OS) of the entire group was 57 months, and the 1-and 2-year OS rates were 64.6% and 56.0%. The median progress free survival(PFS) was 11 months, and the 1-and 2-year PFS rates were 41.3% and 27.6%, respectively. Patients in the chidamide + CHOP/CHOEP group showed a trend of superior survival compared with patients in the CHOP/CHOEP group, but there was no significant difference(P=0.135). Twenty-five patients(73.5%) were positive for whole blood EBV-DNA test or pathological tissue EBER test. The OS of patients with positive EBV-DNA at diagnosis was worse than those with negative EBV-DNA(P=0.034). However, EBER status had no significant effect on survival. Conclusion: AITL often occurs in the elderly patients, which mostly onset at advanced stage. Autoimmune phenomenon is relatively common. Most AITL patients are positive for whole blood EBV-DNA or pathological tissue EBER test. Positive EBV-DNA is related to the poor prognosis of AITL patients. However, EBER status has no significant effect on survival. The efficacy of CHOP/CHOEP regimen is not satisfactory in AITL patients. Chidamide combined with chemotherapy may improve the efficacy and long-term survival of AITL patients.
作者
魏冲
张炎
王为
张薇
周道斌
WEI Chong;ZHANG Yan;WANG Wei;ZHANG Wei;ZHOU Daobin(Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing,100730,China)
出处
《临床血液学杂志》
CAS
2021年第3期149-155,共7页
Journal of Clinical Hematology
基金
国家自然科学基金(No:81970188)
中国医学科学院医学与健康科技创新工程(No:2016-12M-1-001)。
作者简介
通信作者:周道斌,E-mail:zhoudb@pumch.cn。
