Published in:
01-09-2021 | Epstein-Barr Virus | case report
Death of unknown cause? Post-mortem diagnosis of fulminant course of an EBV-associated secondary hemophagocytic lymphohistiocytosis
Authors:
Josia Fauser, Stefan Köck, Eberhard Gunsilius, Andreas Chott, Andreas Peer, Adelheid Ditlbacher, Gernot Fritsche, Michael Joannidis, Dominik Wolf, Andreas Pircher, M.D., Ph.D.
Published in:
memo - Magazine of European Medical Oncology
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Issue 3/2021
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Summary
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.