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Hemophagocytic lymphohistiocytosis in an adult patient with super-refractory status epilepticus.
Haanpää, Anna; Kämppi, Leena; Kantonen, Jonas; Myllykangas, Liisa; Laakso, Sini M; Forss, Nina.
Afiliación
  • Haanpää A; Department of Neurology, Neurocenter, Helsinki University Hospital, Helsinki, Finland.
  • Kämppi L; Department of Clinical Neurosciences, University of Helsinki, Helsinki, Finland.
  • Kantonen J; Department of Neurology, Neurocenter, Helsinki University Hospital, Helsinki, Finland.
  • Myllykangas L; Department of Clinical Neurosciences, University of Helsinki, Helsinki, Finland.
  • Laakso SM; Epilepsia Helsinki, Member of EpiCare ERN, Helsinki, Finland.
  • Forss N; Department of Pathology, University of Helsinki, Helsinki, Finland.
Epilepsia Open ; 9(5): 1962-1967, 2024 Oct.
Article en En | MEDLINE | ID: mdl-39151036
ABSTRACT
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Estado Epiléptico / Linfohistiocitosis Hemofagocítica Límite: Adult / Humans / Male Idioma: En Revista: Epilepsia Open Año: 2024 Tipo del documento: Article País de afiliación: Finlandia Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Estado Epiléptico / Linfohistiocitosis Hemofagocítica Límite: Adult / Humans / Male Idioma: En Revista: Epilepsia Open Año: 2024 Tipo del documento: Article País de afiliación: Finlandia Pais de publicación: Estados Unidos