Cytokine storm syndrome (CSS), which is frequently fatal, has garnered increased attention with the ongoing coronavirus pandemic. A variety of hyperinflammatory conditions associated with multiorgan system failure can be lumped under the CSS umbrella, including familial hemophagocytic lymphohistiocytosis (HLH) and secondary HLH associated with infections, hematologic malignancies, and autoimmune and autoinflammatory disorders, in which case CSS is termed macrophage activation syndrome (MAS). Various classification and diagnostic CSS criteria exist and include clinical, laboratory, pathologic, and genetic features. Familial HLH results from cytolytic homozygous genetic defects in the perforin pathway employed by cytotoxic CD8 T lymphocytes and natural killer (NK) cells. Similarly, NK cell dysfunction is often present in secondary HLH and MAS, and heterozygous mutations in familial HLH genes are frequently present. Targeting overly active lymphocytes and macrophages with etoposide and glucocorticoids is the standard for treating HLH; however, more targeted and safer anticytokine (e.g., anti-interleukin-1, -6) approaches are gaining traction as effective alternatives.

中文翻译：

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细胞因子风暴综合症

细胞因子风暴综合症（CSS）通常是致命的，随着当前的冠状病毒大流行，它引起了越来越多的关注。与多器官系统衰竭相关的各种高炎症性疾病可以归为 CSS 范畴，包括家族性噬血细胞性淋巴组织细胞增多症 (HLH) 和与感染、血液恶性肿瘤以及自身免疫和自身炎症性疾病相关的继发性 HLH，在这种情况下 CSS 被称为巨噬细胞活化综合征（马航）。存在各种分类和诊断 CSS 标准，包括临床、实验室、病理和遗传特征。家族性 HLH 是由细胞毒性 CD8 T 淋巴细胞和自然杀伤 (NK) 细胞所使用的穿孔素途径中的溶细胞纯合性遗传缺陷引起的。同样，继发性 HLH 和 MAS 中经常存在 NK 细胞功能障碍，并且家族性 HLH 基因中经常存在杂合突变。使用依托泊苷和糖皮质激素靶向过度活跃的淋巴细胞和巨噬细胞是治疗 HLH 的标准；然而，更具针对性和更安全的抗细胞因子（例如抗白细胞介素-1、-6）方法作为有效的替代方案正在受到关注。