原发性肺结外鼻型NK/T细胞淋巴瘤一例临床分析

目的报道国内第一例肺部原发性结外鼻型NK/T淋巴瘤,分析原发性肺鼻型NK/T淋巴瘤的临床特点和诊断治疗。方法通过1例病理证实的原发性肺结外鼻型淋巴瘤的病例分析,结合文献,对原发性肺NK/T淋巴瘤的免疫表型、发病机制、临床特点、影像学表现、诊断、治疗及预后进行分析。结果NK/T细胞淋巴瘤是一种新近认识的恶性淋巴瘤,原发于肺部的比较罕见,具有特殊的免疫表型与临床病理特点,EB病毒在其发病中扮演重要角色。此瘤恶性程度很高,疾病进展快,治疗效果及预后差。结论原发性肺结外鼻型NK/T淋巴瘤临床罕见,诊断难度大,预后差,目前缺少有效的治疗方法。

Primary pulmonary Extranodal nasal-type NK/T-cell lymphoma:report of one case and a brief review of the literature

Objective To report the first case of primary pulmonary extranodal nasal-type NK/T-cell lymphoma in China,and to update the relevant literature.Methods One case of primary pulmonary extranodal nasal-type NK/T-cell lymphoma confirmed by pathology was presented.We reviewed immunophenotype,pathogenesis,clinical feature,imaging manifestation,diagnosis,treatment and prognosis of the disease.Results Extranodal nasal-type NK/T-cell lymphoma is a newly acknowledged disease with a rapid clinical progress and rarely originated in lung.It owns special immunophenotype and clinical pathological features.It was aggressive with a poor outcome.Epstein-Barr virus plays a very important role in pathogenesis.Conclusions Primary pulmonary extranodal nasal-type NK/T-cell lymphoma is extremely rare with poor prognosis and difficult to diagnose.There is no efficient therapy presently.