A subset of patients with severe COVID-19 develop profound inflammation and multi-organ dysfunction consistent with a “Cytokine Storm Syndrome” (CSS). In this review we compare the clinical features, diagnosis, and pathogenesis of COVID-CSS with other hematological CSS, namely secondary hemophagocytic lymphohistiocytosis (sHLH), idiopathic multicentric Castleman disease (iMCD), and CAR-T cell therapy associated Cytokine Release Syndrome (CRS). Novel therapeutics targeting cytokines or inhibiting cell signaling pathways have now become the mainstay of treatment in these CSS. We review the evidence for cytokine blockade and attenuation in these known CSS as well as the emerging literature and clinical trials pertaining to COVID-CSS. Established markers of inflammation as well as cytokine levels are compared and contrasted between these four entities in order to establish a foundation for future diagnostic criteria of COVID-CSS.

部分重症 COVID-19 患者会出现严重的炎症和多器官功能障碍，与“细胞因子风暴综合征”(CSS) 一致。在这篇综述中，我们将 COVID-CSS 的临床特征、诊断和发病机制与其他血液学 CSS 进行比较，即继发性噬血细胞性淋巴组织细胞增多症 (sHLH)、特发性多中心 Castleman 病 (iMCD) 和 CAR-T 细胞治疗相关细胞因子释放综合征 (CRS) ）。针对细胞因子或抑制细胞信号传导途径的新型疗法现已成为这些 CSS 的主要治疗方法。我们回顾了这些已知 CSS 中细胞因子阻断和减弱的证据以及与 COVID-CSS 相关的新兴文献和临床试验。对这四个实体之间已建立的炎症标记物以及细胞因子水平进行比较和对比，以便为未来的 COVID-CSS 诊断标准奠定基础。