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

Josia Fauser; Stefan Köck; Eberhard Gunsilius; Andreas Chott; Andreas Peer; Adelheid Ditlbacher; Gernot Fritsche; Michael Joannidis; Dominik Wolf; Andreas Pircher

doi:10.1007/s12254-021-00701-9

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

Memo. 2021;14(3):287-291. doi: 10.1007/s12254-021-00701-9. Epub 2021 Apr 1.

Affiliations

¹ Department of Hematology and Oncology, Internal Medicine V, Medical University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.
² Institute of Pathology and Microbiology, Wilheminenspital, Vienna, Austria.
³ Division of Intensive Care and Emergency Medicine, Department of Internal Medicine, Medical University of Innsbruck, Innsbruck, Austria.
⁴ Department of Internal Medicine II, Medical University of Innsbruck, Innsbruck, Austria.

Abstract

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.

Keywords: Epstein–Barr virus; Fever of unknown origin; HScore; Hemophagocytic lymphohistiocytosis; Hemophagocytosis.