Epstein–Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8–9 September 2008
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Abstract
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.
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.
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.
The participants proposed an international consortium to facilitate further clinical
and biological studies of novel EBV-driven LPDs.