Fatal Epstein-Barr virus infection in a 63-year-old man. An autopsy report

A 63-year-old man with acute, heterophil-negative Epstein-Barr (EBV) viral infection displayed neurologic impairment that progressed to coma and death. Fever, pharyngitis, and lymphadenopathy were notably absent. There was no lymphocytosis, and multiple peripheral smears revealed few atypical lymphocytes. Results of specific EBV serology were diagnostic of acute infection. At the time of autopsy, there was massive intravascular and perivascular infiltration of all organs by lymphocytes and atypical mononuclear cells. There was depletion of the paracortical T-lymphocyte areas of lymph nodes. The atypical mononuclear cells did not contain intracytoplasmic immunoglobulin, as shown by the immunoperoxidase technique, nor did they take up esterase stains, but their electron-microscopic features were characteristic of lymphoid cells. These morphologic findings suggest a T-cell defect, with unrestricted proliferation of B lymphocytes. The lack of characteristic clinical and hematologic features in this case underscores the value of specific EBV serology in the diagnosis of acute heterophil-negative EBV infection.