Annals of Oncology Advance Access published online on June 10, 2009
Annals of Oncology, doi:10.1093/annonc/mdp064
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Epstein–Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8–9 September 2008
1 Medical Virology Section, Laboratory of Clinical Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
2 Department of Virology, Nagoya University Graduate School of Medicine, Nagoya
3 Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Showa-ku, Japan
4 Department of Pathology, Samsung Medical Center, Seoul, Korea
5 Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
* Correspondence to: Dr E. S. Jaffe, Hematopathology Section and Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Building 10/Room 2B 42, 10 Center Drive MSC-1500, Bethesda, MD 20892-1500, USA. Tel: +1-301-496-0183; Fax: +1-301-402-2415; E-mail: elainejaffe{at}nih.gov
Background: Recently novel Epstein–Barr virus (EBV) lymphoproliferative diseases (LPDs) have been identified in non-immunocompromised hosts, both in Asia and Western countries. These include aggressive T-cell and NK-cell LPDs often subsumed under the heading of chronic active Epstein–Barr virus (CAEBV) infection and EBV-driven B-cell LPDs mainly affecting the elderly.
Design: To better define the pathogenesis, classification, and treatment of these disorders, participants from Asia, The Americas, Europe, and Australia presented clinical and experimental data at an international meeting.
Results: The term systemic EBV-positive T-cell LPD, as adopted by the WHO classification, is preferred as a pathological classification over CAEBV (the favored clinical term) for those cases that are clonal. The disease has an aggressive clinical course, but may arise in the background of CAEBV. Hydroa vacciniforme (HV) and HV-like lymphoma represent a spectrum of clonal EBV-positive T-cell LPDs, which have a more protracted clinical course; spontaneous regression may occur in adult life. Severe mosquito bite allergy is a related syndrome usually of NK cell origin. Immune senescence in the elderly is associated with both reactive and neoplastic EBV-driven LPDs, including EBV-positive diffuse large B-cell lymphomas.
Conclusion: The participants proposed an international consortium to facilitate further clinical and biological studies of novel EBV-driven LPDs.
chronic active EBV infection, diffuse large B-cell lymphoma, hemophagocytic syndrome, hydroa vacciniforme, immune senescence, senile EBV-positive lymphoproliferative disease, systemic EBV-positive lymphoproliferative disease
Received for publication December 22, 2008. Revision received February 16, 2009. Accepted for publication February 18, 2009.