Death of unknown cause? Post-mortem diagnosis of fulminant course of an EBV-associated secondary hemophagocytic lymphohistiocytosis.

HLH is a life-threatening disease, which is characterized by a dysregulated immune response with uncontrolled T cell and macrophage activation. The often fulminant course of the disease needs a fast diagnostic work-up to initiate as soon as possible the appropriate therapy. We present herein the case of a 71-year-old patient with rapidly progressive hyperinflammatory syndrome, which post mortem resulted in the diagnosis of EBV-associated HLH. With this case report, we intend to highlight the relevance of the HScore in the diagnosis of HLH, to create a greater awareness for EBV as a trigger of HLH, and to demonstrate the importance of treating EBV-associated HLH as early as possible.

Informations de copyright

© The Author(s) 2021.

Déclaration de conflit d'intérêts

Conflict of interestJ. Fauser, S. Köck, E. Gunsilius, A. Chott, A. Peer, A. Ditlbacher, G. Fritsche, M. Joannidis, D. Wolf, and A. Pircher declare that they have no competing interests.