Background: Hemophagocytic syndrome (HPS) is clinically defined as a combination of fever, liver dysfunction,\r\ncoagulation abnormalities, pancytopenia, progressive macrophage proliferation throughout the reticuloendothelial\r\nsystem, and cytokine over-production, and may be primary or secondary to infectious, auto-immune, and tumoral\r\ndiseases. The most consistent association is with viral infections but, as it is still debated whether any\r\nmicro-organisms are involved in its pathogenesis, we critically appraised the literature concerning HPS and its\r\nrelationship with infections.\r\nDiscussion: Infection-dependent HPS has been widely observed, but there are no data concerning its incidence in\r\nchildren. A better understanding of the pathophysiology of HPS may clarify the interactions between the immune\r\nsystem and the variously implicated potential infectious agents. Epstein-Barr virus (EBV) infection has been\r\nprominently associated with HPS, with clonal proliferation and the hyperactivation of EBV-infected T cells. However,\r\na number of other viral, bacterial, fungal, and parasitic infections have been reported in association with HPS. In the\r\ncase of low-risk HPS, corticosteroids and/or intravenous immunoglobulin or cyclosporine A may be sufficient to\r\ncontrol the biological process, but etoposide is recommended as a means of reversing infection-dependent\r\nlymphohistiocytic dysregulation in high-risk cases.\r\nSummary: HPS is a potential complication of various infections. A polymerase chain reaction search for infectious\r\nagents including EBV, cytomegalovirus and Leishmania is recommended in clinical settings characterised by\r\nnon-remitting fever, organomegaly, cytopenia and hyperferritinemia.
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