CELL INTELLIGENCE · 肿瘤细胞治疗研究
肿瘤细胞治疗研究
Emapalumab 用于 CD19 靶向 CAR-T 后免疫效应细胞相关噬血细胞性淋巴组织细胞增生症样综合征的 2 例 B-ALL 患者：临床与生物标志物相关性
英文原题：Emapalumab for Immune Effector Cell-Associated Hemophagocytic Lymphohistiocytosis-Like Syndrome Following CD19-Directed CAR-T in Two Patients With B-ALL: Clinical and Biomarker Correlates.
查看英文原题
Emapalumab for Immune Effector Cell-Associated Hemophagocytic Lymphohistiocytosis-Like Syndrome Following CD19-Directed CAR-T in Two Patients With B-ALL: Clinical and Biomarker Correlates.
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中文摘要
免疫效应细胞相关噬血细胞性淋巴组织细胞增多症样综合征（IEC-HS）是CAR-T 细胞治疗后危及生命的高炎症毒性，与细胞因子释放综合征（CRS）和神经毒性不同。在一项单中心回顾性队列研究中，2023 年 11 月至 2025 年 4 月接受 tisagenlecleucel 治疗的 12 名复发/难治性 B 急性淋巴细胞白血病（B-ALL）儿童及青年患者中，有 2 人（17%）在 CRS 消退后发生 IEC-HS，表现为复发性发热、凝血障碍、肝功能异常、血细胞减少，以及 CXCL9、IL-18 和可溶性 C5b-9 显著升高。使用 emapalumab 治疗后，临床表现和生物标志物迅速改善。这些发现支持 IEC-HS 是一种由干扰素-γ 驱动的综合征，并提示靶向阻断 IFN-γ 可能是有效的治疗策略。
展开英文摘要原文
Immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome (IEC-HS) is a life-threatening hyperinflammatory toxicity distinct from cytokine release syndrome (CRS) and neurotoxicity following chimeric antigen receptor T-cell (CAR-T) therapy.
In a single-institution retrospective cohort of pediatric and young adult patients with relapsed or refractory acute B-lymphoblastic leukemia (B-ALL) treated with tisagenlecleucel between November 2023 and April 2025, two of 12 patients (17%) developed IEC-HS after CRS resolution, marked by recurrent fever, coagulopathy, hepatic dysfunction, cytopenias, and profound elevations in CXCL9, IL-18, and soluble C5b-9. Treatment with emapalumab led to rapid clinical and biomarker improvement.
These findings support IEC-HS as an interferon- -driven syndrome and suggest targeted IFN- blockade as a potentially effective therapeutic strategy.
论文信息
- 作者
- Galletta TJ、Rubinstein JD、Dandoy CE、Khoury R、Faulhaber A、Phillips CL、Krupski C
- 第一作者单位
- Division of Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.United States
- 通讯作者单位
- Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.United States
- 文献类型
- 病例报告
- 期刊
- Pediatric blood & cancer2026 Aug
- 原文标识
- PubMed 42136039 · DOI 10.1002/1545-5017.70406