Nasal NK/T-cell lymphoma: epidemiology and pathogenesis

Abstract

Nasal NK/T-cell lymphoma (NKTCL) is an uncommon disease, but usually shows a highly aggressive clinical course. The disease is much more frequent in Asian and Latin American countries than in Western countries, and is universally associated with Epstein-Barr virus (EBV) infection. Analyses of gene mutations, especially p53 and c-KIT, revealed the different frequencies by district. Epidemiological studies revealed the changes of the disease frequency in Korea during the period from 1977-1989 to 1990-1996. Case-control study showed that the exposure to pesticides and chemical solvents could be causative of NKTCL. Further studies including HLA antigen typing of patients is necessary to further clarify the disease mechanism.

Fig. 1

HE. Polymorphous pattern of proliferation in the nasal cavity. Large cells show positive immunoreactivity with CD3ε, TIA-1, and CD56. ABC method, ×400

Fig. 2

In situ hybridization with EBER-1 probe reveals positive signals in the nucleus of proliferating cell

Fig. 3

Summary of the FAS gene mutations found in patients with nasal NKTCL. The shaded rectangles at the COOH-terminus of the protein represents the small peptide added because of the frameshift in the gene encoding FAS.TM: transmembrane domain

Fig. 4

The mouse WR19L cell line expressing recombinant human FAS protein with (T1102C, A978G, 1095 ins A) or without (wild type) mutations were incubated with various concentrations of anti-FAS antibody at 37.0°C for 16 h. Clones expressing FAS receptor with any mutations (A978G, 1095 ins A, T1102C) were resistant to apoptosis induced by the anti-FAS antibody