Takahara Miki, Kumai Takumi, Kishibe Kan, Nagato Toshihiro, Harabuchi Yasuaki
Department of Otolaryngology-Head and Neck Surgery, Asahikawa Medical University, Asahikawa 078-8510, Japan.
Department of Innovative Head & Neck Cancer Research and Treatment (IHNCRT), Asahikawa Medical University, Asahikawa 078-8510, Japan.
Microorganisms. 2021 Jun 25;9(7):1381. doi: 10.3390/microorganisms9071381.
Extranodal NK/T-Cell Lymphoma, nasal type (ENKTL-NT) has some salient aspects. The lymphoma is commonly seen in Eastern Asia, has progressive necrotic lesions in the nasal cavity, makes midfacial destructive lesions, and shows poor prognosis. The lymphoma cell is originated from either NK- or γδ T-cells, which express CD56. Since the authors first demonstrated the existence of Epstein-Barr virus (EBV) DNA and EBV oncogenic proteins in lymphoma cells, ENKTL-NT has been recognized as an EBV-associated malignancy. Because the angiocentric and polymorphous lymphoma cells are mixed with inflammatory cells on a necrotic background, the diagnosis of ENKTL-NT requires CD56 immunostaining and EBER in situ hybridization. In addition, serum the EBV DNA level is useful for the diagnosis and monitoring of ENKTL-NT. Although ENKTL-NT is refractory lymphoma, the prognosis is improved by the development of therapies such as concomitant chemoradiotherapy. The basic research reveals that a wide variety of intracellular/cell surface molecules, cytokines, chemokines, and micro RNAs are involved in lymphomagenesis, and some of them are related to EBV. Understanding lymphoma behavior introduces new therapeutic strategies, such as the usage of immune checkpoint inhibitors, peptide vaccines, and molecular targeting therapy. This review addresses recent advances in basic and clinical aspects of ENKTL-NT, especially its relation to EBV features.
鼻型结外NK/T细胞淋巴瘤（ENKTL-NT）具有一些显著特点。该淋巴瘤常见于东亚地区，鼻腔内有进行性坏死性病变，可导致面部中部破坏性病变，且预后较差。淋巴瘤细胞起源于NK细胞或γδT细胞，表达CD56。自作者首次在淋巴瘤细胞中证实存在爱泼斯坦-巴尔病毒（EBV）DNA和EBV致癌蛋白以来，ENKTL-NT已被认为是一种与EBV相关的恶性肿瘤。由于以坏死为背景的血管中心性和多形性淋巴瘤细胞与炎性细胞混合存在，ENKTL-NT的诊断需要进行CD56免疫染色和EBER原位杂交。此外，血清EBV DNA水平对ENKTL-NT的诊断和监测有帮助。尽管ENKTL-NT是难治性淋巴瘤，但伴随放化疗等治疗方法的发展使其预后有所改善。基础研究表明，多种细胞内/细胞表面分子、细胞因子、趋化因子和微小RNA参与淋巴瘤的发生，其中一些与EBV有关。对淋巴瘤行为的了解带来了新的治疗策略，如免疫检查点抑制剂、肽疫苗和分子靶向治疗的应用。本文综述了ENKTL-NT基础和临床方面的最新进展，尤其是其与EBV特征的关系。
