T-cell intravascular lymphomatosis (angiotropic large cell lymphoma): association with Epstein-Barr viral infection

Aims: Intravascular lymphomatosis (IVL) is a very rare non-Hodgkin's lymphoma characterized by proliferation of lymphoma cells in the vascular lumina without involvement of adjacent parenchymal tissue. IVL is predominantly of B-cell lineage, but occasional cases of T lineage IVL involving almost exclusively the skin have been described. A case of IVL that occurred initially in the epididymis of a patient with an antecedent nasopharyngeal carcinoma was studied to define the clinicopathological features associated with this unique presentation. Methods and results: This lymphoma was studied by standard histological and immunophenotyping methods. The results showed lymphoma cells confined within the blood vessels, which expressed leucocyte common antigen, and T-cell markers CD3 and UCHL-1. The T-cell origin of the IVL prompted investigations for an assciation with Epstein-Barr virus infection (EBV). Insitu hybridization with digoxigenin-labelled anti-sense RNA probes to EBV encoded RNA (EBER) showed strong signals in the nuclei of virtually all of the lymphoma cells. Conclusions: EBV infection of the malignant cells was demonstrated by in-situ hybridization. This case suggests that T-cell IVL may be another EBV related human neoplasm. This observation will need to be validated by further studies.