Overview and problematic standpoints of severe chronic active Epstein-Barr virus infection syndrome

Epstein-Barr virus (EBV) is an ubiquitous human herpesvirus. Its infection is generally subclinical. However, in certain circumstances, EBV causes infectious mononucleosis (IM) and lymphoproliferative disorders (LPD) in immunologically compromised individuals. Furthermore, EBV infection is etiologically linked to human malignancies such as Burkitt's lymphoma (BL), nasopharyngeal carcinoma (NPC) and miscellaneous malignant diseases because of the presence of viral genome in their tumor tissues. Since the late 1970s, a chronic undefined illness possibly associated with EBV infection, named such as severe chronic active EBV infection syndrome (SCAEBV), has been of interest due to its unique manifestations that often result in a poor prognosis. This review is an overview of SCAEBV with respect to its; history, diagnosis, pathogenesis, therapeutic approaches, and ideas on how to further recognize this enigmatic disease.