Epstein-Barr virus-associated hemophagocytic syndrome.
A virus-associated hemophagocytic syndrome (VAHS) is a non-neoplastic, generalized histiocytic proliferation with prominent hemophagocytosis associated with a systemic viral infection. Epstein-Barr virus (EBV) is one candidate for this association but serologic and molecular biologic studies have been lacking in many cases. Although VAHS is generally a benign process, EBV-associated hemophagocytic syndrome (EBV-AHS) is often fatal and has a relatively high mortality rate. Therefore, EBV-AHS must be distinguished from VAHS caused by other viruses. Recent evidence indicates that the pathophysiology in EBV-AHS appears to be mediated by the unrestricted release of cytokines produced by the EBV-infected T cells. Clinical and laboratory findings, the differential diagnosis, virology studies, pathophysiology, and treatment in EBV-AHS are reviewed.
Kikuta
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Pediatric Clinic, Aiiku Hospital, Children's Aiiku Foundation, Sapporo, Japan.
D000328:Adult; D000914:Antibodies, Viral / Q000032:analysis; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006566:Herpesviridae Infections / Q000150:complications*; D004854:Herpesvirus 4, Human / Q000276:immunology / Q000302:isolation & purification / Q000472:pathogenicity*; D006644:Histiocytes / Q000473:pathology / Q000821:virology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000175:diagnosis / Q000401:mortality / Q000821:virology*; D006801:Humans; D007223:Infant; D008297:Male; D010587:Phagocytosis; D011230:Precancerous Conditions / Q000821:virology; D014412:Tumor Virus Infections / Q000150:complications*
D000914:Antibodies, Viral