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Hemophagocytic lymphohistiocytosis in COVID-19: Case reports of a stepwise approach
Sebastian Schnaubelt1, Daniel Tihanyi2, Robert Strassl3
1Department of Emergency Medicine, Medical University of Vienna.
Insights
Severe COVID-19 can cause secondary hemophagocytic lymphohistiocytosis (sHLH). Early HScore screening and a treatment combining corticosteroids, immunoglobulins, and anakinra may improve outcomes for these critical patients.
Area of Science:
- Critical care medicine
- Immunology
- Infectious diseases
Background:
- Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection can trigger a hyperinflammatory state.
- The immunologic syndrome in severe COVID-19 often mimics secondary hemophagocytic lymphohistiocytosis (sHLH).
- Optimal management strategies for COVID-19 patients with sHLH remain unclear.
Observation:
- Three critically ill COVID-19 patients with hyperinflammation and cytokine storm requiring mechanical ventilation were reviewed.
- Diagnosis of sHLH was confirmed using the HScore.
- A treatment regimen involving methylprednisolone, pentaglobin, and anakinra was administered.
Findings:
- One patient survived intensive care.
- Two patients diagnosed late with sHLH unfortunately deceased.
- Routine HScore screening in deteriorating COVID-19 patients is feasible and identifies high-risk individuals.
Implications:
- Early identification and intervention for sHLH in COVID-19 may improve patient survival.
- A stepwise therapeutic approach including corticosteroids, immunoglobulins, anakinra, and immunoadsorption can mitigate cytokine storm effects.
- Further research is warranted to optimize sHLH management in the context of COVID-19.
Rationale:
The immunologic syndrome induced by severe acute coronavirus disease 2019 (COVID-19) is yet not fully understood. Typical patterns of clinical and laboratory features match secondary hemophagocytic lymphohistiocytosis (sHLH). However, the optimal approach to COVID-19 patients testing positive for sHLH is still unclear.
Patient Concerns:
Three patients with COVID-19 are reviewed. All showed hyperinflammation and cytokine storm, necessitating intensive care treatment including mechanical ventilation.
Diagnosis:
Secondary hemophagocytic lymphohistiocytosis due to severe COVID-19; diagnosed via HScore.
Interventions:
A treatment regimen of methylprednisolone, pentaglobin, and anakinra was developed and administered.
Outcomes:
One patient survived the ICU stay. Two other patients, in whom sHLH was diagnosed too late, deceased.
Lessons:
A routine screening of COVID-19 patients for secondary HLH by using the HScore is feasible; especially those patients deteriorating clinically with no sufficient response to shock management might be at particular high risk. A stepwise therapeutic approach comprising corticosteroids, immunoglobulins and anakinra, accompanied by immunoadsorption, may dampen cytokine storm effects, and potentially reduce mortality.
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