NK／T细胞鼻型淋巴瘤研究进展

Recent Advances on Extranodal NK/T-cell Lymphoma of Nasal Type——Review

NK/T细胞鼻型淋巴瘤(extranodal NK/T-cell lymphoma,ENKL)是2001年WH0淋巴造血组织肿瘤新分类中的一个独立类型,较多见于亚洲东部以及南美地区,好发于成年男性,中位年龄50岁。ENKL多发生于结外,主要表现为面部中线结构的毁损性破坏,最常见的部位是鼻和腭部,病程表现具有极高的侵袭性。发病机制尚不完全清楚,与EB病毒感染关系密切。基本组织学改变是凝固性坏死和多种炎细胞混合浸润的背景上肿瘤性淋巴样细胞散在或弥漫分布。瘤细胞表达NK细胞相关抗原如CD2、CD56、胞浆CD3ε、CD16、CD43、CD94,表达细胞毒性颗粒相关蛋白。原位杂交检测大部分EBER呈阳性。病情易于复发且对治疗耐药,因此ENKL患者整体预后很差。近年来很多研究致力于ENKL的治疗,但是最佳治疗措施尚不肯定。早期患者多采用放疗后联合化疗;晚期ENKL预后很差,各种化疗方案治疗往往无效,以左旋门冬酰胺为基础的化疗可能是较有希望的新的解救化疗方案,造血干细胞移植可能是一种有效的治疗方法。病程进展迅速,预后很差,需积极寻找不良预后相关因素。本文就ENKL的病因及发病机制、临床表现与分期、病理特征、诊断及鉴别诊断、治疗及预后的研究进展进行了综述。

Extranodal NK/T-cell lymphoma, nasal type （ENKT）, is a distinct clinicopathologic disease, common among East Asia and Latin America population. Clinically, it frequently occurs in middle-aged men. It predominantly occurs in the nasal or paranasal areas and less frequently in the skin. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. The pathogenesis of EN- KT remains uncertain. It is thought that Epstein-Barr virus （EBV） may play a role in the development of this entity. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. It has unique characteristics including the expression of cytoplasmic CD3, CD56 and cytotoxic molecules such as TIA-1, and is positive for EBV in situ hybridization. The overall prognosis of this disease is poor because of frequent relapse or resistance to treatment. Although several studies have explored the treatment of ENKL in recent years, the optimal therapy has still not been found. Due to the highly aggressive features of tumors, every endeavor has been made to investigate factors associated with poor outcome. In this review, the recent advances on cause and pathogenesis, clinical manifestations and staging, pathologic characteristics, diagnosis and differential diagnosis, therapy and prognosis of ENKL are summarized.