Fatal atypical T-cell proliferation associated with Epstein-Barr virus infection

We report the case of a young Caucasian man who presented with polyneuropat hy and severe, ultimately fatal, congestive heart failure in the context of a chronic active Epstein-Barr virus (EBV) infection. Post-mortem examinati on revealed both monoclonal and polyclonal proliferation of EBV-positive at ypical T lymphocytes within different organs. Predominant infiltration of t he nervous system and heart with extensive myocardial scarring accounted fo r the clinical symptoms. The remarkable features of this case are (i) the o ccurrence in a Caucasian patient, (ii) the absence of detectable immunodefi ciency, and (iii) the myocardial destruction by EBV-infected monoclonal T c ells.