Alan Ozkan, Bulbul Mustafa Cem, Enlice Mehmet Ali, Mandel Nil Molinas
Department of Internal Medicine, Division of Medical Oncology, Koc University Hospital, Istanbul, Türkiye.
Department of Internal Medicine, Division of Medical Oncology, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Türkiye.
Immunotherapy. 2025 Jan;17(1):11-17. doi: 10.1080/1750743X.2025.2451604. Epub 2025 Jan 15.
Hemophagocytic Lymphohistiocytosis (HLH) is a severe and potentially life-threatening condition characterized by an excessive and uncontrolled activation of the immune system. ICI-related hemophagocytic lymphohistiocytosis (irHLH) is a rare immune-related adverse event with an incidence of 0.03% to 0.4%. Although rare, it can be potentially lethal, with a high mortality rate of up to 50% in some cases. We present a patient with recurrent glioblastoma who developed Hemophagocytic Lymphohistiocytosis s a result of nivolumab treatment and was subsequently managed with cytokine-directed therapy (tocilizumab). Early diagnosis and treatment of Hemophagocytic Lymphohistiocytosis (HLH) associated with immune checkpoint inhibitors (ICIs) are indeed crucial due to the potentially life-threatening nature of the condition.Cytokine-based treatments (such as anti-IL-6) may be appropriate for patients who do not respond to high-dose steroids.
噬血细胞性淋巴组织细胞增生症（HLH）是一种严重且可能危及生命的疾病，其特征为免疫系统过度且不受控制的激活。免疫检查点抑制剂相关的噬血细胞性淋巴组织细胞增生症（irHLH）是一种罕见的免疫相关不良事件，发病率为0.03%至0.4%。尽管罕见，但它可能具有潜在致命性，在某些情况下死亡率高达50%。我们报告了一名复发性胶质母细胞瘤患者，该患者因纳武利尤单抗治疗而发生噬血细胞性淋巴组织细胞增生症，随后接受了细胞因子导向治疗（托珠单抗）。由于噬血细胞性淋巴组织细胞增生症（HLH）与免疫检查点抑制剂（ICIs）相关的病情具有潜在威胁生命的性质，因此早期诊断和治疗确实至关重要。基于细胞因子的治疗（如抗IL-6）可能适用于对大剂量类固醇无反应的患者。
