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Do COVID-19 Infections Result in a Different Form of Secondary Hemophagocytic Lymphohistiocytosis
Raymond Chu1, Charmaine van Eeden2, Sneha Suresh3
1Division of Rheumatology, Department of Medicine, The Ottawa Hospital, University of Ottawa, Ottawa, ON K1H 7W9, Canada.
Insights
Severe COVID-19 may involve a cytokine storm similar to hemophagocytic lymphohistiocytosis (HLH). Further research is needed to confirm COVID-19 associated HLH and explore targeted immunosuppression benefits.
Area of Science:
- Immunology
- Infectious Diseases
- Hematology
Background:
- The COVID-19 pandemic presents a global health crisis with high morbidity and mortality.
- Severe cases of COVID-19 are linked to a cytokine storm, resembling hemophagocytic lymphohistiocytosis (HLH).
- This similarity suggests potential therapeutic benefits from immunosuppression in severe COVID-19.
Purpose of the Study:
- To review the literature on HLH diagnosis and features, focusing on secondary HLH.
- To identify knowledge gaps concerning a potential COVID-19 associated HLH.
- To evaluate the utility of current diagnostic criteria and novel biomarkers for identifying COVID-19 HLH-like presentations.
Main Methods:
- Literature review of hemophagocytic lymphohistiocytosis (HLH) and COVID-19.
- Analysis of diagnostic criteria for HLH (e.g., HScore, HLH-2004).
- Exploration of potential biomarkers (e.g., soluble CD163, NK cell activity) for COVID-19 associated HLH.
Main Results:
- Existing HLH diagnostic criteria may not be optimal for identifying COVID-19 associated HLH.
- A COVID-19 associated HLH entity requires further clarification.
- Novel biomarkers might offer improved diagnostic accuracy for COVID-19 HLH-like conditions.
Conclusions:
- The precise nature of HLH in severe COVID-19 remains unclear.
- Current diagnostic tools may need refinement for COVID-19 patients.
- Further investigation into biomarkers is crucial for understanding and managing severe COVID-19.
Abstract:
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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