Berning Philipp, Ngoya Maud, Kim Won Seog, Shumilov Evgenii, Wu Depei, Huang Haiwen, Cairoli Anne, Tucci Alessandra, Dachy Guillaume, Gounot Romain, Wilke Anne C, Scheid Christof, Dreger Peter, Lopez Lorenzo Jose Luis, Bloor Adrian, Romejko-Jarosinska Joanna, Gadisseur Alain, Schroers Roland, Reményi Péter, Gabellier Ludovic, Poiani Monica, Halahleh Khalid, Galimard Jacques-Emmanuel, Lenz Georg, Sureda Anna, Bazarbachi Ali, Glass Bertram, Schmitz Norbert
Department of Medicine A, Hematology, Oncology and Pneumology University Hospital Muenster Muenster Germany.
European Society for Blood and Marrow Transplantation Paris France.
Hemasphere. 2025 Aug 15;9(8):e70184. doi: 10.1002/hem3.70184. eCollection 2025 Aug.
Natural killer (NK)/T-cell lymphomas (NKTCLs) are rare, aggressive lymphomas prevalent in East Asia and South America. Despite improvements, largely due to asparaginase-based therapies, outcomes for advanced disease remain poor, and the role of autologous stem cell transplantation (auto-HCT) remains controversial. This study evaluated real-world outcomes of auto-HCT in NKTCL patients across Asia and Europe. We included 130 adult NKTCL patients undergoing auto-HCT between 2011 and 2022 using data from the European Society for Blood and Marrow Transplantation (EBMT) registry and South Korean registry data. Median age was 51.3 years; 66.9% were male. Most patients (95.1%) had an Eastern Cooperative Oncology Group (ECOG) score 0-1; 65.3% had Stage III-IV disease. One prior therapy line was reported in 53.1%, and ≥2 lines in 46.9%. Asparaginase-based regimens were used in 79.5% pretransplant. Responses at auto-HCT included complete (59.7%), partial (27.9%) remission, and stable/progressive disease (12.4%). Epstein-Barr virus (EBV)-DNA in the peripheral blood was reported in 37.3%. With a median follow-up of 4.6 years, 3-year overall survival (OS) and progression-free survival (PFS) were 63.8% and 47.6%. Relapse and NRM rates at 3 years were 46.7% and 5.7%. Patients in complete remission had improved 3-year OS (75.2%) compared to PR (52.8%) and stable/progressive disease (32.0%) (P = 0.007). Detectable EBV-DNA in the blood at auto-HCT was associated with poor outcomes (3-year OS: 26.7% vs. 78.1% in patients with undetectable EBV-DNA; P < 0.0001). Patients achieving complete remission and undetectable EBV-DNA in the blood before auto-HCT had a favorable survival, suggesting auto-HCT may be a treatment option in selected high-risk patients. This is the largest multinational cohort evaluating prognostic factors for auto-HCT for NKTCL.
自然杀伤（NK）/T细胞淋巴瘤（NKTCL）是一种罕见的侵袭性淋巴瘤，在东亚和南美洲较为常见。尽管在很大程度上由于基于天冬酰胺酶的治疗方法有所改善，但晚期疾病的治疗效果仍然很差，自体干细胞移植（auto-HCT）的作用仍存在争议。本研究评估了亚洲和欧洲NKTCL患者自体造血干细胞移植的实际疗效。我们纳入了2011年至2022年间接受自体造血干细胞移植的130例成年NKTCL患者，数据来自欧洲血液和骨髓移植学会（EBMT）登记处以及韩国登记处的数据。中位年龄为51.3岁；66.9%为男性。大多数患者（95.1%）东部肿瘤协作组（ECOG）评分为0-1；65.3%为III-IV期疾病。53.1%的患者曾接受过一线治疗，46.9%的患者接受过≥2线治疗。79.5%的患者在移植前使用了基于天冬酰胺酶的方案。自体造血干细胞移植后的缓解情况包括完全缓解（59.7%）、部分缓解（27.9%）以及病情稳定/进展（12.4%）。外周血中报告有EB病毒（EBV）-DNA的患者占37.3%。中位随访4.6年，3年总生存率（OS）和无进展生存率（PFS）分别为63.8%和47.6%。3年复发率和非复发死亡率分别为46.7%和5.7%。完全缓解的患者3年总生存率（75.2%）高于部分缓解（52.8%）和病情稳定/进展（32.0%）的患者（P = 0.007）。自体造血干细胞移植时血液中可检测到EBV-DNA与不良预后相关（3年总生存率：可检测到EBV-DNA的患者为26.7%，未检测到EBV-DNA的患者为78.1%；P < 0.0001）。在自体造血干细胞移植前达到完全缓解且血液中未检测到EBV-DNA的患者生存率良好，这表明自体造血干细胞移植可能是部分高危患者的一种治疗选择。这是评估NKTCL自体造血干细胞移植预后因素的最大规模跨国队列研究。
