Scholarlink

<p>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 (<i>P</i> = 0.009) but did not affect overall survival (OS) (<i>P</i> = 0.306), whereas asparaginase-containing lymphoma regimens significantly improved both OS (<i>P</i> = 0.025) and 60-day survival rates (<i>P</i> = 0.016) in treatment-naive patients. By multivariate analysis, significant independent predictors of 60-day poor outcomes included serum albumin level &lt; 30&#xa0;g/L (HR, 2.03; 95% CI, 1.08–3.83; <i>P</i> = 0.029), platelet count &lt; 20 × 10⁹/L (HR, 2.70; 95% CI, 1.46–4.99; <i>P</i> = 0.002), and Epstein-Barr virus DNA (whole blood) &gt; 33,850 copies/mL (HR, 1.97; 95% CI, 1.01–3.81; <i>P</i> = 0.045). ECOG performance status ≥ 2 (HR, 2.00; 95% CI, 1.24–3.23; <i>P</i> = 0.004) and platelet count &lt; 20 × 10⁹/L (HR, 7.61; 95% CI, 2.14–27.09; <i>P</i> = 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.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

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)

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年1月30日

查看全文
  • Yongle Li,
  • Yanping Liu,
  • Yilian Yang,
  • Zihan Liang,
  • Yi Xia,
  • Ling Gao,
  • Jingxin Zhou,
  • Bingzong Li,
  • Chunling Wang,
  • Liang Yu,
  • Miao Sun,
  • Yuqing Miao,
  • Haiwen Ni,
  • Xiaoyan Xie,
  • Yunping Zhang,
  • Min Zhao,
  • Guoqiang Lin,
  • Tao Jia,
  • Qiudan Shen,
  • Lei Fan,
  • Jianyong Li,
  • Xuzhang Lu,
  • Wenyu Shi,
  • Yi Miao

摘要

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 × 10⁹/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 × 10⁹/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.