Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening state of immune hyperactivation that arises in the setting of genetic mutations and infectious, inflammatory, or neoplastic triggers. Sustained, aberrant activation of cytotoxic CD8⁺ T cells and resultant inflammatory cytokine release are core pathogenic mechanisms. Key clinical features include high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. HLH is likely under-recognized, and mortality remains high, especially in adults; thus, prompt diagnosis and treatment are essential. Familial forms of HLH are currently treated with chemotherapy as a bridge to hematopoietic stem cell transplantation. HLH occurring in rheumatic disease (macrophage activation syndrome) is treated with glucocorticoids, IL-1 blockade, or cyclosporine A. In other forms of HLH, addressing the underlying trigger is essential. There remains a pressing need for more sensitive, context-specific diagnostic tools. Safer, more effective therapies will arise with improved understanding of the cellular and molecular mechanisms of HLH.

噬血细胞淋巴组织细胞增生症（HLH）是一种罕见的威胁生命的免疫过度激活状态，它发生在遗传突变和传染性，炎性或肿瘤性触发因素的背景下。持续，异常激活细胞毒性CD8 ⁺T细胞和导致的炎性细胞因子释放是核心致病机制。主要临床特征包括持续高烧，肝脾肿大，血细胞减少，转氨酶和铁蛋白水平升高以及凝血病。HLH可能未被充分认识，并且死亡率仍然很高，尤其是在成年人中；因此，及时诊断和治疗至关重要。HLH的家族形式目前已通过化学疗法作为造血干细胞移植的桥梁。风湿性疾病（巨噬细胞活化综合征）中发生的HLH用糖皮质激素，IL-1阻滞剂或环孢霉素A治疗。在其他形式的HLH中，解决潜在的触发因素至关重要。迫切需要更敏感的，特定于上下文的诊断工具。更安全