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与鼻型结外 NK/T 细胞淋巴瘤或侵袭性 NK 细胞白血病相关的噬血细胞性淋巴组织细胞增生症:江苏省淋巴瘤协作组 (JCLG) 的多中心回顾性研究

英文原题:Hemophagocytic lymphohistiocytosis associated with extranodal NK/T cell lymphoma, nasal type or aggressive NK cell leukemia: a retrospective multicenter study of Jiangsu Cooperative Lymphoma Group (JCLG).

查看英文原题

Hemophagocytic lymphohistiocytosis associated with extranodal NK/T cell lymphoma, nasal type or aggressive NK cell leukemia: a retrospective multicenter study of Jiangsu Cooperative Lymphoma Group (JCLG).

PubMed 2026/01/30(内容时间) Ann Hematol Q3 · IF 2.3(JCR 2025)

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中文摘要

噬血细胞性淋巴组织细胞增多症(HLH)是一种危及生命的高炎症综合征,其中NK细胞恶性肿瘤相关HLH较为罕见,尚未得到系统研究。本项多中心回顾性队列研究分析了2010至2024年间136例成年NK细胞恶性肿瘤相关HLH患者数据。

结果显示,初始接受含依托泊苷的HLH治疗可改善60天生存率(P=0.009),但不影响总生存期(OS;P=0.306);而对于既往未经治疗的患者,含门冬酰胺酶的淋巴瘤治疗方案可显著改善OS(P=0.025)和60天生存率(P=0.016)。多变量分析显示,60天结局不良的显著独立预测因素包括血清白蛋白<30 g/L(HR=2.03;95% CI 1.08–3.83;P=0.029)、血小板计数<20×10⁹/L(HR=2.70;95% CI 1.46–4.99;P=0.002)以及EB病毒DNA(全血)>33,850 copies/mL(HR=1.97;95% CI 1.01–3.81;P=0.045)。ECOG体能状态评分2分(HR=2.00;95% CI 1.24–3.23;P=0.004)和血小板计数<20×10⁹/L(HR=7.61;95% CI 2.14–27.09;P=0.002)是OS不良的独立危险因素。

因此,我们建议既往未经治疗的成年NK细胞恶性肿瘤相关HLH患者一线采用含门冬酰胺酶的方案。未来需要多中心前瞻性研究,以优化含门冬酰胺酶方案、评估新型联合策略,并建立精准风险预测模型指导临床实践。

展开英文摘要原文

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome, among which NK-cell malignancy-associated HLH represents a clinically rare entity that has not been systematically investigated. The multicenter retrospective cohort study analyzed data from 136 adult patients diagnosed with HLH related to NK-cell malignancies between 2010 and 2024. The results demonstrated that initial treatment with etoposide-containing HLH therapy improved 60-day survival rates (P = 0. 009) but did not affect overall survival (OS) (P = 0. 306), whereas asparaginase-containing lymphoma regimens significantly improved both OS (P = 0.

025) and 60-day survival rates (P = 0. 016) in treatment-naive patients. By multivariate analysis, significant independent predictors of 60-day poor outcomes included serum albumin level < 30 g/L (HR, 2. 03; 95% CI, 1. 08-3. 83; P = 0. 029), platelet count < 20 10 /L (HR, 2. 70; 95% CI, 1. 46-4. 99; P = 0.

002), and Epstein-Barr virus DNA (whole blood) > 33,850 copies/mL (HR, 1. 97; 95% CI, 1. 01-3. 81; P = 0. 045). ECOG performance status 2 (HR, 2. 00; 95% CI, 1. 24-3. 23; P = 0. 004) and platelet count < 20 10 /L (HR, 7. 61; 95% CI, 2. 14-27. 09; P = 0. 002) were independent risk factors for poor OS.

Therefore, we recommend asparaginase-based regimens as first-line therapy for treatment-naive adult patients with HLH related to NK-cell malignancies. Future multicenter prospective studies are warranted to optimize asparaginase-containing regimens, evaluate novel combination strategies, and establish precision risk-prediction models to guide clinical practice.

论文信息

作者
Li Y、Liu Y、Yang Y、Liang Z、Xia Y、Gao L、Zhou J、Li B
第一作者单位
Department of Hematology, The First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.China
通讯作者单位
Department of Hematology, The First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China. miaoyi1992@hotmail.com.China
文献类型
多中心研究
期刊
Annals of hematology2026 Jan 30
原文标识
PubMed 41612048 · DOI 10.1007/s00277-026-06847-y