Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States

Abstract

Chronic active EBV disease (CAEBV) is

Epstein-Barr Virus: A Spectrum of Disease from Mononucleosis to Lymphoma

“…Another fascinating aspect of the many links between the human immune system and EBV is the occurrence of chronic active EBV infections most common in Japan but also seen sporadically in other geographical areas (10,11). These infections often occur in childhood and are characterized by the atypical infection of T and NK lymphocytes and may culminate in lymphoma.…”

Unusual Indolent Course of a Chronic Active Epstein-Barr Virus-Associated Natural Killer Cell Lymphoproliferative Disorder

“…3,7 The diagnostic criteria for this disease entity was first proposed by Okano et al ulceration/proliferation, coronary artery aneurysms, central nervous system involvement, myocarditis and interstitial pneumonia. 11 In addition, these patients respond poorly to chemotherapy and have a high risk of eventual progression to overt leukaemia and lymphomas in the form of extranodal NK/T cell lymphomas of nasal type (ENKLs), aggressive NK cell leukaemia (ANKL) and peripheral T cell leukaemia or lymphomas.…”

EBV-Associated T-Cell Lymphoproliferative Diseases in Young Adults with Unusual Histopathological and Immunophenotypic Features

“…CAEBV is found to be prevalent in Asian countries and characterized by proliferation of the EBV-infected T/NK cells which is related with EBV-associated T/NK-cell lymphoproliferative disorders (EBV + T/NK-LPDs) [5], however, CAEBV is mostly associated with EBV-infected B cells in Western countries [6]. CAEBV of T/NK type shows a broad range of clinical manifestations from indolent, localized forms to a more systemic, aggressive form with the types of the infected cells clonality ranging from polyclonal to monoclonal, which are different from case to case, as do the prognoses [7] [8].…”