Division of Hematology, Department of Medicine, Maine Medical Center, Portland, ME 04102, USA.
Division of Hematology-Oncology, Department of Medicine, Monter Cancer Center, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Lake Success, NY 11042, USA.
Exp Biol Med (Maywood). 2022 Feb;247(4):330-337. doi: 10.1177/15353702211068840. Epub 2022 Jan 22.
Cytokine storm is an umbrella term that describes an inflammatory syndrome characterized by elevated levels of circulating cytokines and hyperactivation of innate and/or adaptive immune cells. One type of cytokine storm is hemophagocytic lymphohistiocytosis (HLH), which can be either primary or secondary. Severe COVID-19-associated pneumonia and acute respiratory distress syndrome (ARDS) can also lead to cytokine storm/cytokine release syndrome (CS/CRS) and, more rarely, meet criteria for the diagnosis of secondary HLH. Here, we review the immunobiology of primary and secondary HLH and examine whether COVID-19-associated CS/CRS can be discriminated from non-COVID-19 secondary HLH. Finally, we review differences in immunobiology between these different entities, which may inform both clinical diagnosis and treatment of patients.
细胞因子风暴是一个总称，用于描述一种炎症综合征，其特征是循环细胞因子水平升高，固有免疫和/或适应性免疫细胞过度激活。细胞因子风暴的一种类型是噬血细胞性淋巴组织细胞增生症（HLH），可分为原发性或继发性。严重的 COVID-19 相关肺炎和急性呼吸窘迫综合征（ARDS）也可导致细胞因子风暴/细胞因子释放综合征（CS/CRS），且更罕见地符合继发性 HLH 的诊断标准。在这里，我们回顾原发性和继发性 HLH 的免疫生物学，并探讨 COVID-19 相关 CS/CRS 是否可与非 COVID-19 继发性 HLH 相区分。最后，我们还比较了这些不同实体之间的免疫生物学差异，这可能有助于临床诊断和治疗患者。
