Hemophagocytic lymphohistiocytosis in COVID-19: Case reports of a stepwise approach.

Abstract

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.

• Authors: Sebastian Schnaubelt, Daniel Tihanyi, Robert Strassl, Ralf Schmidt, Sonja Anders, Anton N Laggner, Hermine Agis, Hans Domanovits
• Journal: Medicine (Medicine (Baltimore)) Vol. 100 Issue 12 Pg. e25170 (Mar 26 2021) ISSN: 1536-5964 [Electronic] United States
• PMID: 33761694 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

Chemical References

• Anti-Inflammatory Agents
• Immunoglobulin A
• Immunoglobulin M
• Immunoglobulins, Intravenous
• Immunosuppressive Agents
• Interleukin 1 Receptor Antagonist Protein
• pentaglobulin
• Methylprednisolone

Topics

• Aged
• Anti-Inflammatory Agents (therapeutic use)
• COVID-19 (complications, physiopathology, therapy)
• Combined Modality Therapy
• Critical Care
• Cytokine Release Syndrome (drug therapy)
• Delayed Diagnosis
• Fatal Outcome
• Female
• Humans
• Immunoglobulin A (therapeutic use)
• Immunoglobulin M (therapeutic use)
• Immunoglobulins, Intravenous (therapeutic use)
• Immunosuppressive Agents (therapeutic use)
• Interleukin 1 Receptor Antagonist Protein (therapeutic use)
• Lymphohistiocytosis, Hemophagocytic (diagnosis, drug therapy, etiology)
• Male
• Methylprednisolone (therapeutic use)
• Middle Aged
• Respiration, Artificial
• SARS-CoV-2