Abstract
Nasal NK/T-cell lymphoma (NKTCL) frequently presents with necrotic, granulomatous lesions in the upper respiratory tract, and usually shows a highly aggressive clinical course. Thus, it was initially included in the clinical condition of lethal midline granuloma. Recently, the disease has been recognized as a neoplastic proliferation of NK/T cells. 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. Abnormalities of other genes have also been reported. Case-control studies 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.
Highlights
Rapid destruction of the nose and face by necrotic and granulomatous lesions was first described by McBride[1]
It was shown that proliferating cells in polymorphic reticulosis (PR) had large granular lymphocyte morphology[5], which is characteristic of natural killer (NK) cells or cytotoxic T lymphocytes
High-resolution genetic typing revealed a significantly lower frequency of HLA-A*0201 in NK/T-cell lymphoma (NKTCL) than in the normal population[22]. These findings suggest that HLA-A*0201–restricted cytotoxic T lymphocyte (CTL) responses may function in vivo to suppress the development of NKTCL, or in other words, the role of Epstein-Barr virus (EBV) for NKTCL development
Summary
Lymphoma arises from clonal expansion of lymphoid cells that are transformed by the accumulation of genetic lesions affecting oncogenes and tumor-suppressor genes. The amount of samples from NKTCL lesions available for genetic analyses is small and samples usually contain massive necrotic areas. Abnormalities of p53, k-ras, c-kit, and β-catenin genes in NKTCL cases were examined by polymerase chain reaction (PCR) – single strand conformation polymorphism (SSCP) followed by a direct sequence method. In the studies for NKTCL in Asia, exons 4-8 or exons 5-8, which encompass about 90% of mutations reported in the literature[25], were examined in one institute (Table 1). Shenyang is in northern China, adjacent to the Korean peninsula, suggesting that environmental and genetic factors might generate the differences in frequency
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