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 fo;this association but serologic and molecular biolo gic studies have been lacking in many cases. Although VAHS is generall y a benign process, EBV-associated hemophagocytic syndrome (EBV-AHS) i s often fatal and has a relatively high mortality rate. Therefore, EBV -AHS must be distinguished from VAHS caused by other viruses. Recent e vidence indicates that the pathophysiology in EBV-AHS appears to be me diated by the unrestricted release of cytokines produced by the EBV-in fected T cells. Clinical and laboratory findings, the differential dia gnosis, virology studies, pathophysiology, and treatment in EBV-AHS ar e reviewed.