Is COVID-19-associated cytokine storm distinct from non-COVID-19 secondary hemophagocytic lymphohistiocytosis?

Authors

Johnson M. Liu, Division of Hematology, Department of Medicine, Maine Medical Center, Portland, ME 04102, USA.
Jeffrey Chi, 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.

Document Type

Article

Publication Date

2-1-2022

Journal Title

Experimental biology and medicine (Maywood, N.J.)

MeSH Headings

Antibodies, Monoclonal (therapeutic use); Antibodies, Neutralizing (therapeutic use); COVID-19 (complications); Cytokine Release Syndrome (etiology, therapy, virology); Humans; Interleukin 1 Receptor Antagonist Protein (therapeutic use); Lymphohistiocytosis, Hemophagocytic (diagnosis, etiology, immunology, therapy)

Abstract

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.

First Page

330

Last Page

337

Recommended Citation

Liu JM, Chi J. Is COVID-19-associated cytokine storm distinct from non-COVID-19 secondary hemophagocytic lymphohistiocytosis?. Exp Biol Med (Maywood). 2022;247(4):330-337. doi:10.1177/15353702211068840