Issue: 2003 > November > case report

Chronic active Epstein-Barr virus infection in an adult with no detectable immune deficiency

M. de Boer, M.J.T.M. Mol, M.J.J.T. Bogman, J.M.D. Galama, R.A.P. Raymakers


Introduction: Epstein-Barr virus (EBV) establishes lifelong latent infection. In some patients the host-virus balance is disturbed, resulting in a chronic active EBV infection. The following case illustrates the difficulty in diagnosing and treating chronic EBV infection.
Case: A 30-year-old woman was referred because of
recurrent swellings of lymphatic tissue of both eyelids,
orbit and lymph nodes and general malaise since the
age of 19. In the past, repeated biopsies showed MALT
lymphoma and nonspecific lymphoid infiltrations. Now,
a biopsy of an axillary lymph node showed paracortical
hyperplasia with a polymorphous polyclonal lymphoid
proliferation, and large numbers of EBV-encoded small
RNA (EBER) positive cells, consistent with EBV infection.
Laboratory investigation showed a high EBV viral load.
No evidence of immunodeficiency was found. Chronic
active EBV infection (CAEBV) was diagnosed. Treatment
with high-dose acyclovir did not significantly reduce the
viral load. Rituximab was given in an attempt to reduce
the amount of EBV-infected B lymphocytes. However,
soon after the second dose the patient died of a subarachnoidal haemorrhage.
Conclusion: This case report illustrates CAEBV as a rare
manifestation of EBV-induced disease, which will be
detected more frequently with the use of EBV-EBER
hybridisation of lymph nodes and polymerase chain
reaction (PCR) for EBV DNA. The prognosis is poor
with no established therapeutic strategies.