García-Cosío Mónica, Santón Almudena, Méndez M Carmen, Rivas Carmen, Martín Carmen, Bellas Carmen
Department of Pathology, Ramón y Cajal Hospital, Madrid, Spain.
Tumori. 2003 May-Jun;89(3):278-84. doi: 10.1177/030089160308900309.
Lymphoid malignancies expressing CD56 are rare and most occur in the nasal or nasopharyngeal region. They derive from natural killer cells or from a small subset of T cells that have granular cytoplasm containing molecules that mediate cytotoxic activity: TIA-1, granzyme B and perforin. Both types are closely associated with Epstein-Barr virus.
We report the pathologic, immunophenotypic and molecular findings in 14 cases of nasopharyngeal/nasal type T/NK lymphomas.
Clinically, all patients had localized disease and also had symptoms limited to the nose. The neoplastic cells were frequently pleomorphic, and angiocentric growth was common. Combined immunophenotypic and gene rearrangement analyses demonstrated that most of the cases were true NK cell tumors and were either CD56+ and CD3- or CD56+ and CD3+. Immunohistochemical study showed TIA-1 and granzyme B expression in all cases. By in situ hybridization, most of the cases were associated to Epstein-Barr virus, harboring type 1 virus, and polymerase chain reaction amplification across the 30 bp deletion showed high frequency of latent membrane protein-1-deleted variants.
The nasal type T/NK cell lymphoma shows distinctive clinicopathologic, immunophenotypic and molecular features. These results confirm the important role of Epstein-Barr virus as a local factor in their pathogenesis.
表达CD56的淋巴系统恶性肿瘤较为罕见，多数发生于鼻腔或鼻咽部。它们起源于自然杀伤细胞或一小部分具有颗粒状细胞质且含有介导细胞毒性活性分子（TIA-1、颗粒酶B和穿孔素）的T细胞。这两种类型均与爱泼斯坦-巴尔病毒密切相关。
我们报告了14例鼻咽/鼻型T/NK淋巴瘤的病理、免疫表型及分子学研究结果。
临床上，所有患者均为局限性病变，且症状局限于鼻部。肿瘤细胞常呈多形性，血管中心性生长常见。联合免疫表型及基因重排分析显示，多数病例为真正的自然杀伤细胞肿瘤，要么CD56阳性且CD3阴性，要么CD56阳性且CD3阳性。免疫组织化学研究显示所有病例均有TIA-1和颗粒酶B表达。通过原位杂交，多数病例与爱泼斯坦-巴尔病毒相关，携带1型病毒，且跨越30 bp缺失的聚合酶链反应扩增显示潜伏膜蛋白-1缺失变异体的高频率。
鼻型T/NK细胞淋巴瘤具有独特的临床病理、免疫表型及分子学特征。这些结果证实了爱泼斯坦-巴尔病毒作为其发病机制中的局部因素的重要作用。
