Epstein-Barr Virus (EBV) in Extranodal T-cell Non-Hodgkin's Lymphomas (T-NHL). Identification of Nasal T-NHL as a Distinct Clinicopathological Entity Associated with EBV

Abstract

T-cell Non-Hodgkin's lymphomas (T-NHL) can be defined as clonal malignant proliferations related phenotypically and functionally to normal T-cell populations of the lymphoid tissue. There is increasing evidence that T-NHL with similar morphology but originating from different sites differ in their clinical behaviour, immunophenotypic features, oncogene expression and relation with oncogenic viruses such as HTLV-I and EBV. Indeed, it has been shown that the prevalence of EBV in T-NHL is related to the site of origin. Thus, EBV was found in nearly all nasal T-NHL but only in a proportion of primary nodal, lung, gastrointestinal and Waldeyer's ring T-NHL while it was undetectable in most primary cutaneous T-NHL. Besides their constant association with EBV, nasal T-NHL display peculiar clinical, histological, immunophenotypic and genotypic features. They present clinically as lethal midline granuloma and histologically as pleomorphic malignant tumours variably associated with angiocentricity, angioinvasion and necrosis. Moreover, they frequently exhibit extensive loss of T-cell antigens, including CD3 and TCRαβ and γδ proteins, usually express the Natural Killer (NK)-related CD56 antigen and frequently show absence of clonal rearrangements of TCR β, γ and δ loci. Therefore, among T-NHL, nasal T-NHL can be regarded as a distinct clinicopathologic entity associated with EBV, which could be derived either from immature T-cells or from NK cells.

Key Words:

• EBV
• Extranodal lymphoma
• T-cell lymphomas
• nasal T-lymphoma