Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008.
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.


METHODS
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.


CONCLUSIONS
The participants proposed an international consortium to facilitate further clinical and biological studies of novel EBV-driven LPDs.
Cohen
J I
JI
Medical Virology Section, Laboratory of Clinical Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Kimura
H
H
Department of Virology, Nagoya University Graduate School of Medicine, Nagoya.
Nakamura
S
S
Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Showa-ku, Japan.
Ko
Y-H
YH
Department of Pathology, Samsung Medical Center, Seoul, Korea.
Jaffe
E S
ES
Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. Electronic address: elainejaffe@nih.gov.
D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000276:immunology* / Q000628:therapy; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000276:immunology* / Q000628:therapy / Q000821:virology*