[Research advance on Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis]

Abstract

Epstein-Barr virus (EBV) is the major triggering factor producing virus-associated hemophagocytic syndrome (VAHS). The clinical manifestations were various and it's diagnosis should meet HLH-2004 criteria as well as EBV-positive, and familial hemophagocytic lymphohistiocytosis and lymphoma-associated hemophagocytic syndrome must be ruled out, too. In terms of treatment, combination of dexamethasone, VP-16 and cyclosporin A is the first choice, if they do not work, hematopoietic stem cell transplantation can be done. In this review, the recent advance on pathogenesis, clinical features, diagnostic problems, treatment strategies and prognosis of EBV-HLH have been described.