Epstein-Barr 病毒阳性 NK/T 细胞恶性肿瘤在波兰：独特特征、治疗策略与生存洞察——波兰淋巴瘤研究组的真实世界回顾性报告

A retrospective analysis of 23 patients diagnosed between 2018 and 2023 across six Polish centers was conducted (20 ENKTCL and three ANKL).

Mean age was 50.1 years, with 73.9% male. Early stages and low-risk dominated (75%). Common first-line treatments included DeVIC (41%) and asparaginase-based regimens (45%). Rituximab was used in three first-line and two second-line cases. Consolidation with autologous hematopoietic cell transplantation was performed in four and three cases, respectively. The 2-year progression-free survival (PFS) and overall survival (OS) were 57.8% and 75.2%, respectively. Early disease stage and response to the first-line therapy were associated with higher PFS with hazard ratios (HRs) 3.5 (95% confidence interval [CI]: 1.6-8.97) and 1.84 (95% CI: 1.02-3.3), respectively. Hemophagocytic lymphohistiocytosis (HLH) was found in 21.7% of patients and was the strongest predictor of poor OS with HR 10.79 (95% CI: 1.96-59.5).

The demographic and clinical characteristics of the Polish patient population were similar to those reported in Asian populations. Approximately 45% of patients received asparaginase-containing regimens. The efficacy of first-line regimens seemed to be lower compared to those reported in the literature. The presence of HLH is the most critical factor predicting poor survival.