Abstract
Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative diseases (EBV-T/NK-LPDs) are a group of rare diseases resulting from ectopic infection of T or natural killer (NK) lymphocytes with Epstein-Barr virus (EBV). EBV-T/NK-LPDs include chronic active EBV infection, EBV-associated hemophagocytic lymphohistiocytosis, hydroa vacciniforme-like lymphoproliferative disease, and severe mosquito bite allergy. Extra-nodal NK/T-cell lymphoma-nasal type and aggressive NK-cell leukemia can also be included in this broad spectrum. Currently, the etiology of EBV-T/NK-LPDs is unknown and no curative therapy has been established, except for hematopoietic stem cell transplantation. While most cases of EBV-T/NK-LPDs have been documented in specific areas of the world, they have also been documented more broadly across East Asia and Latin America. Consequently, active research and discussion of EBV-T/NK-LPDs are both necessary and important within the extensive international community of scientists and clinicians, to elucidate their etiology and develop a standard therapy.
Highlights
Epstein-Barr virus (EBV) is a ubiquitous gammaherpesvirus that persistently infects more than 90% of the world’s adult population
Infection with EBV is usually asymptomatic, the development of symptomatic disease has been associated with a delayed primary infection leading to infectious mononucleosis in adolescents and young adults, and with various EBV-associated malignancies
EBV can induce chronic disease with prolonged infectious mononucleosis-like symptoms and a sustained EBV DNA load in the peripheral blood. This disease has been called chronic active EBV disease or chronic active EBV infection; both are abbreviated as CAEBV [1, 2]
Summary
Epstein-Barr virus (EBV) is a ubiquitous gammaherpesvirus that persistently infects more than 90% of the world’s adult population. EBV can induce chronic disease with prolonged infectious mononucleosis-like symptoms and a sustained EBV DNA load in the peripheral blood Rare, this disease has been called chronic active EBV disease or chronic active EBV infection; both are abbreviated as CAEBV [1, 2]. In addition to CAEBV, there is a group of rare diseases that develop in the apparent absence of immunodeficiency and are characterized by ectopic infection of EBV in T or NK lymphocytes They are EBV-associated hemophagocytic lymphohistiocytosis (HLH), hydroa vacciniforme (HV)-like lymphoproliferative disease, and severe mosquito bite allergy (SMBA). The ideal timing and protocol for hematopoietic stem cell transplantation for CAEBV remain to be determined This review investigates both original works and secondary sources on EBV-T/NK-LPDs. It is intended to stimulate discussion on the enigmatic pathogenesis and disease concept of EBV-T/NK-LPDs, to facilitate, eventually, the development of a standard therapy
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