肠病相关T细胞淋巴瘤：一项基于荷兰人群的发病率、治疗及预后队列研究。
Enteropathy-associated T-cell lymphoma: A population-based cohort study on incidence, treatment, and outcome in the Netherlands.
作者信息
Meeuwes Frederik O, Brink Mirian, Plattel Wouter J, Vermaat Joost S P, Kersten Marie José, Wondergem Mariëlle, Visser Otto, van der Poel Marjolein W M, Oostvogels Rimke, Woei-A-Jin F J Sherida H, Böhmer Lara, Snijders Tjeerd J F, Huls Gerwin A, Nijland Marcel
机构信息
Department of Hematology University Medical Center Groningen Groningen the Netherlands.
Department of Hematology Medisch Spectrum Twente Enschede the Netherlands.
出版信息
EJHaem. 2024 Nov 29;5(6):1215-1222. doi: 10.1002/jha2.1049. eCollection 2024 Dec.
INTRODUCTION
Enteropathy-associated T-cell lymphoma (EATL) is a peripheral T-cell lymphoma (PTCL) with a poor prognosis. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with or without etoposide consolidated by autologous stem cell transplantation (ASCT) are recommended for fit PTCL patients. The role of etoposide and ASCT in EATL is unclear.
METHODS
This study reports the incidence, treatment, and outcome of EATL patients using the Netherlands Cancer Registry, with nationwide coverage of >95%.
RESULTS
All patients diagnosed in 1989-2021 ( = 351, 77% treated) were identified (median age 67 years, 56% male, 50% limited stage). Time period analysis assessed trends in primary therapy and overall survival (OS). Treatment included chemotherapy (CT) (34%), surgery (18%), surgery and CT (19%) or CT followed by ASCT (7%). The 5-year OS for treated patients with limited versus advanced stage was 19% and 9% respectively. The 2-year OS improved over time (21%-33%, 0.06). Surgery only (hazard ratio [HR] 2.16; 95% confidence interval [CI] 1.55-3.01, 0.01) and advanced-stage disease (HR 1.67; 95% CI 1.25-2.23, 0.01) were predictors of poor prognosis. ASCT (HR 0.31; 95% CI 0.18-0.56) was associated with improved OS.
CONCLUSION
There was no statistical difference in OS between patients treated with or without etoposide. Current first-line treatment is ineffective.
引言
肠病相关T细胞淋巴瘤（EATL）是一种预后较差的外周T细胞淋巴瘤（PTCL）。对于身体状况适合的PTCL患者，推荐使用环磷酰胺、阿霉素、长春新碱和泼尼松（CHOP）方案，联合或不联合依托泊苷，并通过自体干细胞移植（ASCT）进行巩固治疗。依托泊苷和ASCT在EATL中的作用尚不清楚。
方法
本研究利用荷兰癌症登记处报告了EATL患者的发病率、治疗情况和预后，该登记处覆盖全国范围超过95%。
结果
确定了1989年至2021年期间诊断的所有患者（n = 351，77%接受了治疗）（中位年龄67岁，56%为男性，50%为局限期）。时间段分析评估了初始治疗和总生存期（OS）的趋势。治疗方法包括化疗（CT）（34%）、手术（18%）、手术加CT（19%）或CT后行ASCT（7%）。局限期与晚期接受治疗患者的5年总生存率分别为19%和9%。2年总生存率随时间有所提高（21% - 33%，P = 0.06）。单纯手术（风险比[HR] 2.16；95%置信区间[CI] 1.55 - 3.01，P < 0.01）和晚期疾病（HR 1.67；95% CI 1.25 - 2.23，P < 0.01）是预后不良的预测因素。ASCT（HR 0.31；95% CI 0.18 - 0.56）与总生存期改善相关。
结论
接受或未接受依托泊苷治疗的患者在总生存期方面无统计学差异。目前的一线治疗无效。
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