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自然杀伤(NK)/T细胞淋巴瘤(NKTCL)作为非霍奇金淋巴瘤(NHL)的独立类型,在亚洲、拉丁美洲地区发病率明显高于西方国家。其发病与EB病毒(EBV)感染关系密切,而遗传因素、农药、化学溶剂等与其发病有一定相关性。病理组织学上以血管中心和多形淋巴网状内皮细胞的破坏性浸润为特征,表达T或NK细胞抗原,以及T细胞内抗原-1、粒酶B、穿孔素等细胞毒颗粒相关蛋白,组织标本中绝大部分EBV编码的小分子RNA检测阳性。临床上以中轴区受累为多见,早期病变常局限,进展期则呈侵袭性生长、少数可发生远处转移。目前诊断主要依靠组织标本的病理学检查,CD2+、CD3ε+及CD56+可作为诊断该肿瘤较特异的免疫组织化学标记;同时,EBV相关成分及白介素-9、白介素-10和γ-干扰素等相关细胞因子和蛋白的检出对诊断具有积极帮助,卟啉钙黏蛋白15则可望成为诊断NK细胞来源肿瘤的新的重要标记。近年来,影像学检查、尤其18氟标记脱氧葡萄糖正电子发射型计算机断层扫描显像仪对诊断的价值更加受到重视,有利于与B细胞来源淋巴瘤鉴别,并对NKTCL的分期及预后判断有一定价值。
Natural killer (NK) / T-cell lymphoma (NKTCL), an independent category of non-Hodgkin’s lymphoma (NHL), has a significantly higher prevalence in Asia and Latin America than in Western countries. The incidence of Epstein-Barr virus (EBV) infection is closely related to the genetic factors, pesticides, chemical solvents and their pathogenesis have a certain relevance. Histopathological vascular center and pleomorphic lymphatic endothelial cells characterized by destructive invasion of T or NK cell antigen expression, and T cell antigen-1, granzyme B, perforin and other cytotoxic particles-related proteins, The majority of EBV-encoded small-molecule RNAs in tissue samples were positive for detection. Axial area involvement is more common clinical, early lesions are often limited, the progress was invasive growth, a few may occur distant metastasis. At present, the diagnosis mainly depends on the pathological examination of the tissue samples. CD2 +, CD3ε + and CD56 + can be used as specific immunohistochemical markers for the diagnosis of the tumor. Meanwhile, the related components of EBV and the correlation between interleukin-9, interleukin-10 and interferon-γ Detection of cytokines and proteins is helpful in diagnosis. Porphyrin cadherin 15 is expected to become a new important marker in the diagnosis of NK cell-derived tumors. In recent years, imaging examination, especially 18 fluorine-labeled deoxyglucose positron emission computed tomography imaging instrument more attention to the value of diagnosis is conducive to the identification of B-cell lymphoma, and NKTCL staging and prognosis are A certain value.