Hemophagocytic syndrome caused by primary herpes simplex virus 1 infection: report of a first case

Cusini, A. ; Günthard, H. ; Stussi, G. ; Schwarz, U. ; Fehr, T. ; Grueter, E. ; Meerbach, A. ; Bossart, W. ; Schaer, D. ; Rudiger, A.

In: Infection, 2010, vol. 38, no. 5, p. 423-426

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    Summary
    Introduction: Hemophagocytic syndrome represents a severe hyperinflammatory condition by activated macrophages. Leading viral triggering agents are Epstein-Barr virus (EBV), cytomegalovirus (CMV), and adenovirus. Materials and methods: We present a patient with Wegener's granulomatosis on azathioprine and prednisone medication, who developed a life-threatening hemophagocytic syndrome. Positive plasma polymerase chain reaction (PCR) with negative serology revealed a primary, disseminated infection with herpes simplex virus-1 as the triggering pathogen. After treatment with acyclovir, high-dose steroids, immunoglobulins, and etoposide, the patient recovered. Conclusion: Early diagnosis of potentially underlying infections of hemophagocytic syndrome influences the therapeutic approach. It is important to consider a variety of infectious agents, particularly in immunosuppressed individuals. The reported case emphasizes the importance of screening for herpes simplex virus 1