Hemophagocytic syndrome caused by primary herpes simplex virus 1 infection: report of a first case.
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.
Cusini
A
A
Division of Infectious Diseases and Hospital Epidemiology, University Hospital Zurich, Zurich 8091, Switzerland. alexia.cusini@usz.ch
Günthard
H F
HF
Stussi
G
G
Schwarz
U
U
Fehr
T
T
Grueter
E
E
Meerbach
A
A
Bossart
W
W
Schaer
D J
DJ
Rudiger
A
A
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
D013256:Steroids; D005047:Etoposide; D000212:Acyclovir