Abstract: Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is an uncommon lymphoma associated with\r\nthe Epstein-Barr virus (EBV). It most commonly involves the nasal cavity and upper respiratory tract. Primary\r\npulmonary NK/T cell lymphoma is extremely rare. If a patient with a NK or T-cell tumor has an unusual reaction to\r\ntreatment or an unusual prognosis, it is wise to differentiate NK from T-cell tumors. The clinicopathologic\r\ncharacteristics, immunophenotype, EBV in situ hybridization, and T cell receptor (TCR) gene rearrangement of\r\nprimary pulmonary NK cell lymphoma from a 73-year-old Chinese woman were investigated and the clonal status\r\nwas determined using female X-chromosomal inactivation mosaicism and polymorphisms at the phosphoglycerate\r\nkinase (PGK) gene. The lesion showed the typical histopathologic characteristics and immunohistochemical features\r\nof NK/T cell lymphoma. However, the sample was negative for TCR gene rearrangement. A clonality assay\r\ndemonstrated that the lesion was monoclonal. It is concluded that this is the first recorded case of genuine primary\r\npulmonary NK cell lymphoma. The purpose of the present work is to recommend that pathologists carefully\r\ninvestigate the whole lesion to reduce the likelihood that primary pulmonary NK cell lymphoma will be\r\nmisdiagnosed as an infectious lesion. In addition, TCR gene rearrangement and clonal analysis, which is based on\r\nfemale X-chromosomal inactivation mosaicism and polymorphisms at PGK and androgen receptor (AR) loci, were\r\nfound to play important roles in differentiating NK cell lymphoma from T cell lymphoma.\r\nVirtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/\r\nvs/5205300349457729.
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