Severe chronic active EBV infection in an adult patient: case report

Abstract

Severe chronic active Epstein-Barr virus (EBV) infection is a rare and life-threatening illness. Although the criteria for diagnosis include chronic or recurrent infectious mononucleosis-like symptoms lasting more than 6 months and high titers of anti-EBV antibodies, clinical and laboratory findings may be heterogeneous and flexible application of those criteria is necessary in cases showing typical clinical and pathologic findings. We report a case of severe chronic active EBV infection in a 62-yr-old female patient who showed classical clinical findings with infiltration of EBV-infected T lymphocytes in the bone marrow, spleen, and lymph nodes, and died four months after presentation.

Fig. 1

The spleen was markedly enlarged and weighed 685 g. The cut surface showed multifocal necrosis and diffuse congestion.

Fig. 2

Infiltration of lymphoplasma cells and erythrophagocytic histiocytes in spleen (H&E stain, ×400).

Fig. 3

Intranuclear positive signals in lymphoid cells (EBER in situ hybridization ×400).

Fig. 4

CD3-positive T lymphocytes were harboring EBV genome in their nuclei (Double procedure for CD3 immunohistochemistry and EBER in situ hybridization ×400).

Fig. 5

Infiltrated lymphoid cells are predominantly CD4-positive cells (PAP stain, ×400).

Fig. 6

Minority of infiltrated lymphocytes express CD8 (PAP, ×400).