论文 · 病例报告
依马利尤单抗治疗两例B-ALL患者CD19 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：临床与生物标志物关联
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
作者：Thomas J Galletta, Jeremy D Rubinstein, Christopher E Dandoy, Ruby Khoury, Angela Faulhaber, Christine L Phillips, Christa Krupski
Pediatr Blood Cancer · 2026年5月14日 · Galletta 等 7 位作者
不需要生物学背景，多打比方
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摘要Abstract
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-γ-driven syndrome and suggest targeted IFN-γ blockade as a potentially effective therapeutic strategy.
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