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Abstract
Epstein-Barr virus (EBV) is the major triggering factor producing hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis (HLH). In this review, diagnostic problems,
clinical and histopathological features, and treatment strategies of EBV-HLH have
been described. In patients with EBV-HLH, the EBV-infected T cells or natural killer
(NK) cells are mostly mono- or oligo-clonally proliferating, where hypercytokinemia
plays a major role and causes hemophagocytosis, cellular damage and dysfunction of
various organs. Although the majority of EBV-HLH cases develop in apparently immunocompetent
children and adolescents, it also occurs in association with infectious mononucleosis,
chronic active EBV infection, familial HLH, X-linked lymphoproliferative disease,
lymphoproliferative disease like peripheral T-cell lymphoma and NK cell leukemia.
In terms of treatment, special therapeutic measures are required to control the cytokine
storm generated by EBV and to suppress proliferating EBV-genome-containing cells,
because the clinical courses are often fulminant and result in a poor outcome.