Nasal T-cell lymphoma

Abstract

Nasal T-cell lymphomas represent a controversial subset of malignant lymphomas and include lesions previously termed midline malignant reticulosis, lymphomatoid granulomatosis, and polymorphic reticulosis. Nasal T-cell lymphomas are rare in Western populations and much more prevalent in Asian countries. Clinically, adult males are most often affected. Histologically, an angiocentric infiltrate composed of a spectrum of atypical cells is usually present. Phenotypically, the neoplastic cells lack expression of B-lineage markers, and usually express the T-lineage-associated markers CD2, CD45RO, and CD43; however, they often lack other pan-T-lineage markers. They often express the natural killer marker CD56, but usually lack the natural killer markers CD16 and CD57. Gene rearrangement studies have shown a germline configuration for the antigen receptor genes in the majority of cases. To date, evidence of Epstein-Barr virus has been consistently demonstrated, regardless of the geographic region studied. In situ hybridization studies have localized the Epstein-Barr virus to the atypical cells.