title: Hemophagocytic syndrome caused by primary herpes simplex virus 1 infection: report of a first case.
abstract: BACKGROUND
  Hemophagocytic syndrome represents a severe hyperinflammatory condition by activated macrophages. Leading viral triggering agents are Epstein-Barr virus (EBV), cytomegalovirus (CMV), and adenovirus.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Infectious Diseases and Hospital Epidemiology, University Hospital Zurich, Zurich 8091, Switzerland. alexia.cusini@usz.ch
authors.forename: A
authors.initials: A
authors.lastname: Cusini
authors.forename: H F
authors.initials: HF
authors.lastname: Günthard
authors.forename: G
authors.initials: G
authors.lastname: Stussi
authors.forename: U
authors.initials: U
authors.lastname: Schwarz
authors.forename: T
authors.initials: T
authors.lastname: Fehr
authors.forename: E
authors.initials: E
authors.lastname: Grueter
authors.forename: A
authors.initials: A
authors.lastname: Meerbach
authors.forename: W
authors.initials: W
authors.lastname: Bossart
authors.forename: D J
authors.initials: DJ
authors.lastname: Schaer
authors.forename: A
authors.initials: A
authors.lastname: Rudiger
mesh_terms: D000212:Acyclovir / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D006561:Herpes Simplex / Q000188:drug therapy / Q000276:immunology / Q000821:virology*; D018259:Herpesvirus 1, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000821:virology*; D008297:Male; D008875:Middle Aged; D013256:Steroids / Q000627:therapeutic use
chemical_list: D013256:Steroids; D005047:Etoposide; D000212:Acyclovir