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Infection ; 38(5): 423-6, 2010 Oct.
Article in English | MEDLINE | ID: mdl-20602145

ABSTRACT

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.


Subject(s)
Herpes Simplex/virology , Herpesvirus 1, Human/isolation & purification , Lymphohistiocytosis, Hemophagocytic/virology , Acyclovir/therapeutic use , Etoposide/therapeutic use , Herpes Simplex/drug therapy , Herpes Simplex/immunology , Herpesvirus 1, Human/genetics , Humans , Lymphohistiocytosis, Hemophagocytic/drug therapy , Male , Middle Aged , Steroids/therapeutic use
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