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Hemophagocytic lymphohistiocytosis after SARS-CoV-2 vaccination
Marie-Lisa Hieber1, Rosanne Sprute1,2,3, Dennis A Eichenauer1
1Department I of Internal Medicine, Center for Integrated Oncology Aachen Bonn Cologne and Duesseldorf, Faculty of Medicine, University Hospital Cologne, University of Cologne, Cologne, Germany.
Purpose:
The coronavirus disease 2019 (COVID-19) pandemic has led to the approval of novel vaccines with different mechanisms of action. Until now, more than 4.7 billion persons have been vaccinated around the world, and adverse effects not observed in pre-authorization trials are being reported at low frequency.
Methods:
We report a case of severe hemophagocytic lymphohistiocytosis (HLH) after SARS-CoV-2 immunization and performed a literature search for all reported cases of COVID-19 vaccine-associated HLH.
Results:
A 24-year-old female developed HLH after immunization with the mRNA COVID-19 vaccine Comirnaty. Diagnosis was made according to HLH-2004 criteria; the HScore was 259 (> 99% HLH probability) with maximum ferritin of 138.244 µg/L. The patient was initially treated with intravenous immunoglobulins (IVIGs) and dexamethasone without response. The addition of the human interleukin 1 receptor antagonist Anakinra resulted in full recovery within 6 weeks after vaccination. A literature search revealed 15 additional cases of HLH after SARS-CoV-2 vaccination, the majority after immunization with Comirnaty (n = 7) or the viral vector vaccine Vaxzevria (n = 6). Treatment modalities included corticosteroids (n = 13), Anakinra (n = 5), IVIGs (n = 5), and etoposide (n = 2). Eight patients underwent combination treatment. Three of 16 patients died.
Conclusion:
COVID-19 vaccines may occasionally trigger HLH, and Anakinra may be an efficacious treatment option for this condition.
Insights
COVID-19 vaccines can rarely trigger hemophagocytic lymphohistiocytosis (HLH). Anakinra, an IL-1 receptor antagonist, showed efficacy in treating vaccine-associated HLH, leading to full recovery in a severe case.
Area of Science:
- Immunology
- Vaccinology
- Hematology
Background:
- The COVID-19 pandemic spurred rapid development of novel vaccines.
- Post-authorization surveillance has identified rare adverse events.
- Over 4.7 billion individuals have received COVID-19 vaccinations globally.
Observation:
- A case of severe hemophagocytic lymphohistiocytosis (HLH) following mRNA COVID-19 vaccination (Comirnaty) is presented.
- The patient met HLH-2004 criteria with an HScore of 259 and elevated ferritin levels.
- Initial treatment with IVIGs and dexamethasone was ineffective.
Findings:
- A literature review identified 15 additional cases of vaccine-associated HLH.
- Most cases occurred after mRNA (Comirnaty) or viral vector (Vaxzevria) vaccines.
- Anakinra, corticosteroids, IVIGs, and etoposide were used in treatment; Anakinra led to full recovery in the presented case.
- Mortality was observed in 3 out of 16 patients.
Implications:
- COVID-19 vaccines are potential triggers for HLH in susceptible individuals.
- Anakinra represents a promising therapeutic option for managing vaccine-associated HLH.
- Further research into the mechanisms and management of vaccine-induced HLH is warranted.
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