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

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. [ABSTRACT FROM AUTHOR]