Ostrowska Beata, Rymkiewicz Grzegorz, Chechlinska Magdalena, Blachnio Katarzyna, Domanska-Czyz Katarzyna, Bystydzienski Zbigniew, Romejko-Jarosinska Joanna, Borysiuk Anita, Rybski Sebastian, Michalski Wojciech, Walewski Jan
Department of Lymphoid Malignancies, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
Flow Cytometry Laboratory, Department of Pathology and Laboratory Diagnostics, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
Cancers (Basel). 2021 Apr 15;13(8):1911. doi: 10.3390/cancers13081911.
(1) Background: T-cell lymphoblastic lymphoma (T-LBL) is extremely rare and highly aggressive, with no practical risk model defined yet. The prognostic value of T-LBL immunological subtypes is still a matter of controversy. (2) Methods: We re-evaluated 49 subsequent adult T-LBL patients treated according to the German Multicenter Study Group for Adult Acute Lymphoblastic Leukemia (GMALL) protocols, 05/93 ( = 20) and T-LBL 1/2004 ( = 29), 85.7% of which achieved complete remission (CR). (3) Results: The 5/10-year overall survival (OS) and event-free survival (EFS) were 62%/59% and 48%/43%, respectively. In 96% of patients, flow cytometry analyses defining the WHO 2008 immunophenotypes were available. Cortical, early/pro-T/CD2(-), early/pre-T/CD2(+), and mature subtypes were identified in 59.5%, 19%, 15%, and 6.5% of patients, respectively. Overall, 20% of patients had the early T-cell precursor (ETP)-LBL immunophenotype, as proposed by the WHO 2017 classification. For the early/pro-T/CD2(-) subtype, the five-year OS and EFS were 13% and 13%, while for all the other, non-pro-T subtypes, they were 69% and 67%. By multivariate analysis, only CD2(-) status and age > 35 years emerged as strong, independent factors influencing OS and EFS, while the risk of CR failure was influenced by age only (>35 years). (4) Conclusions: ETP was non-significant for OS, unless an ultra-high-risk pro-T/CD2(-) subtype was concerned.
（1）背景：T细胞淋巴母细胞淋巴瘤（T-LBL）极为罕见且侵袭性很强，目前尚无实用的风险模型。T-LBL免疫亚型的预后价值仍存在争议。（2）方法：我们重新评估了49例按照德国成人急性淋巴细胞白血病多中心研究组（GMALL）方案05/93（n = 20）和T-LBL 1/2004（n = 29）治疗的成年T-LBL患者，其中85.7%实现了完全缓解（CR）。（3）结果：5年/10年总生存率（OS）和无事件生存率（EFS）分别为62%/59%和48%/43%。96%的患者可进行定义2008年世界卫生组织免疫表型的流式细胞术分析。分别在59.5%、19%、15%和6.5%的患者中鉴定出皮质型、早期/原T/CD2（-）、早期/前T/CD2（+）和成熟亚型。总体而言，20%的患者具有世界卫生组织2017年分类所提出的早期T细胞前体（ETP）-LBL免疫表型。对于早期/原T/CD2（-）亚型，5年OS和EFS分别为13%和13%，而对于所有其他非原T亚型，它们分别为69%和67%。通过多变量分析，只有CD2（-）状态和年龄> 35岁是影响OS和EFS的强大独立因素，而CR失败风险仅受年龄影响（> 35岁）。（4）结论：除非涉及超高风险的原T/CD2（-）亚型，否则ETP对OS无显著影响。
