The Helmholtz Sino-German Laboratory for Cancer Research, Department of Pathology, Tangdu Hospital, the Fourth Military Medical University, Shaanxi Xi'an 710038, China.
Diagn Pathol. 2013 Aug 19;8:140. doi: 10.1186/1746-1596-8-140.
Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is an uncommon lymphoma associated with the Epstein-Barr virus (EBV). It most commonly involves the nasal cavity and upper respiratory tract. Primary pulmonary NK/T cell lymphoma is extremely rare. If a patient with a NK or T-cell tumor has an unusual reaction to treatment or an unusual prognosis, it is wise to differentiate NK from T-cell tumors. The clinicopathologic characteristics, immunophenotype, EBV in situ hybridization, and T cell receptor (TCR) gene rearrangement of primary pulmonary NK cell lymphoma from a 73-year-old Chinese woman were investigated and the clonal status was determined using female X-chromosomal inactivation mosaicism and polymorphisms at the phosphoglycerate kinase (PGK) gene. The lesion showed the typical histopathologic characteristics and immunohistochemical features of NK/T cell lymphoma. However, the sample was negative for TCR gene rearrangement. A clonality assay demonstrated that the lesion was monoclonal. It is concluded that this is the first recorded case of genuine primary pulmonary NK cell lymphoma. The purpose of the present work is to recommend that pathologists carefully investigate the whole lesion to reduce the likelihood that primary pulmonary NK cell lymphoma will be misdiagnosed as an infectious lesion. In addition, TCR gene rearrangement and clonal analysis, which is based on female X-chromosomal inactivation mosaicism and polymorphisms at PGK and androgen receptor (AR) loci, were found to play important roles in differentiating NK cell lymphoma from T cell lymphoma.
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结外自然杀伤 (NK)/T 细胞淋巴瘤，鼻型，是一种与 EBV 相关的罕见淋巴瘤。它最常累及鼻腔和上呼吸道。原发性肺 NK/T 细胞淋巴瘤极为罕见。如果 NK 或 T 细胞肿瘤患者的治疗反应或预后异常，明智的做法是区分 NK 与 T 细胞肿瘤。研究了一名 73 岁中国女性原发性肺 NK 细胞淋巴瘤的临床病理特征、免疫表型、EBV 原位杂交和 TCR 基因重排，并使用女性 X 染色体失活镶嵌和磷酸甘油酸激酶 (PGK) 基因多态性确定了克隆状态。病变显示 NK/T 细胞淋巴瘤的典型组织病理学特征和免疫组织化学特征。然而，该样本 TCR 基因重排呈阴性。克隆性检测表明病变为单克隆。因此，这是首例确诊的原发性肺 NK 细胞淋巴瘤。本研究的目的是建议病理学家仔细检查整个病变，以降低将原发性肺 NK 细胞淋巴瘤误诊为感染性病变的可能性。此外，TCR 基因重排和基于女性 X 染色体失活镶嵌和 PGK 和雄激素受体 (AR) 基因座多态性的克隆分析对于区分 NK 细胞淋巴瘤和 T 细胞淋巴瘤具有重要作用。
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