Hemophagocytic lymphohistiocytosis in an adult patient with super-refractory status epilepticus.

Autor: Haanpää A; Department of Neurology, Neurocenter, Helsinki University Hospital, Helsinki, Finland.; Department of Clinical Neurosciences, University of Helsinki, Helsinki, Finland., Kämppi L; Department of Neurology, Neurocenter, Helsinki University Hospital, Helsinki, Finland.; Department of Clinical Neurosciences, University of Helsinki, Helsinki, Finland.; Epilepsia Helsinki, Member of EpiCare ERN, Helsinki, Finland., Kantonen J; Department of Pathology, University of Helsinki, Helsinki, Finland., Myllykangas L; Department of Pathology, University of Helsinki, Helsinki, Finland.; Department of Pathology, HUS Diagnostic Center, Helsinki University Hospital, Helsinki, Finland., Laakso SM; Department of Neurology, Neurocenter, Helsinki University Hospital, Helsinki, Finland.; Department of Clinical Neurosciences, University of Helsinki, Helsinki, Finland.; Translational Immunology Research Program, University of Helsinki, Helsinki, Finland., Forss N; Department of Neurology, Neurocenter, Helsinki University Hospital, Helsinki, Finland.; Department of Clinical Neurosciences, University of Helsinki, Helsinki, Finland.
Jazyk: angličtina
Zdroj: Epilepsia open [Epilepsia Open] 2024 Oct; Vol. 9 (5), pp. 1962-1967. Date of Electronic Publication: 2024 Aug 16.
DOI: 10.1002/epi4.13026
Abstrakt: This case report presents a 38-year-old male patient who, after a febrile infection, developed super-refractory status epilepticus and multiorgan failure, and died in 2 weeks despite the best possible intensive care. Autopsy revealed findings suggestive of hemophagocytic lymphohistiocytosis (HLH). This case shows that a rare immunological cause such as HLH may cause febrile infection-related epilepsy syndrome (FIRES), and complications of intensive care can mask the physiological and laboratory changes in HLH. PLAIN LANGUAGE SUMMARY: This case report presents a 38-year-old man who, after a febrile infection, developed intractable epileptic activity requiring intensive care treatment. During the intensive care, the patient showed signs of multiple organ damage and died in 2 weeks despite the best possible treatment. Autopsy revealed findings suggestive of hemophagocytic lymphohistiocytosis (HLH), which is a rare immune system regulation disorder leading to persistent inflammatory state and organ damages. This case shows that an immunological disorder like HLH may underlie treatment resistant fever-related epileptic seizures.
(© 2024 The Author(s). Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)
Databáze: MEDLINE
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