Epstein-Barr virus NK and T cell lymphoproliferative disease: report of a 2018 international meeting

Abstract

Epstein-Barr virus (EBV) normally infects B cells, but in some persons the virus infects T or NK cells. Infection of B cells can result in infectious mononucleosis, and the virus is associated with several B cell malignancies including Hodgkin lymphoma, Burkitt lymphoma, and diffuse large B cell lymphoma. Infection of T or NK cells with EBV is associated with extranodal NK/T cell lymphoma, aggressive NK-cell leukemia, systemic EBV-associated T-cell lymphoma, and chronic active EBV disease, which in some cases can include hydroa vacciniforme-like lymphoproliferative disease and severe mosquito bite allergy. While NK and T cell lymphoproliferative disease is more common in Asia and Latin America, increasing numbers of cases are being reported from the United States and Europe. This review focuses on classification, clinical findings, pathogenesis, and recent genetic advances in NK and T cell lymphoproliferative diseases associated with EBV.

Keywords:

• Aggressive NK-cell leukemia
• chronic active EBV disease
• extranodal NK/T cell lymphoma
• hydroa vacciniforme-like lymphoproliferative disease
• severe mosquito bite allergy
• systemic EBV-positive T-cell lymphoma of childhood

Disclosure statement

Each of the authors reviewed and approved the summary of the work. The authors report no conflict of interest.

Additional information

Funding

This work was supported by the Intramural Research Programs of the National Institute of Allergy and Infectious Diseases, the National Cancer Institute, and the National Human Genome Research Institute. Additional funding was from a generous gift from the Roth Fellowship for CAEBV and Hydroa Vacciniforme.