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Do COVID-19 Infections Result in a Different Form of Secondary Hemophagocytic Lymphohistiocytosis

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Publisher

MDPI
DOI: 10.3390/ijms22062967

Keywords

COVID-19; SARS-CoV-2; natural killer cells; cytokine storm; hemophagocytic lymphohistiocytosis; macrophage activation syndrome

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The COVID-19 pandemic caused by SARS-CoV-2 has raised concerns about a cytokine storm similar to HLH, questioning the effectiveness of current diagnostic criteria. More research is needed to confirm the existence of COVID-19 associated HLH and develop better methods for identification.
The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has resulted in significant morbidity and mortality across the world, with no current effective treatments available. Recent studies suggest the possibility of a cytokine storm associated with severe COVID-19, similar to the biochemical profile seen in hemophagocytic lymphohistiocytosis (HLH), raising the question of possible benefits that could be derived from targeted immunosuppression in severe COVID-19 patients. We reviewed the literature regarding the diagnosis and features of HLH, particularly secondary HLH, and aimed to identify gaps in the literature to truly clarify the existence of a COVID-19 associated HLH. Diagnostic criteria such as HScore or HLH-2004 may have suboptimal performance in identifying COVID-19 HLH-like presentations, and criteria such as soluble CD163, NK cell activity, or other novel biomarkers may be more useful in identifying this entity.

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