中文摘要：

目的：报道以EB病毒相关噬血细胞淋巴组织细胞增生症（EBV-HLH）为最初起病症状的结外鼻型NK/T细胞淋巴瘤1例并进行了文献综述。方法：总结分析以EBV-HLH为最初起病症状的1例成人结外（鼻型）NK/T细胞淋巴瘤的临床资料。详细采集患者病史、体格检查、外周血和骨髓化验、PET-CT影像学及组织病理学等检查资料总结分析,并同时进行文献复习。结果：患者,女,27岁,因＂发热伴咽痛、咳嗽和关节酸痛1月＂入院。入院检查发现全血细胞减少、发热伴肝脾肿大,结合EBV阳性支持EBV-HLH,故予免疫抑制为主的治疗方案,此后患者血常规一度好转。1月后出现带状疱疹,并进行性出现鼻塞。下鼻甲活检病理示浸润细胞CD56＋/-,TIA1＋,GB＋,PF＋,CD3＋,CD43＋,L26-,PAX5-,CD21-,CD30-,KP1-,Ki-67＋（约60%）,EREB＋,诊断为结外鼻型NK/T细胞性淋巴瘤。骨髓像同时找到噬血组织细胞。患者接受了联合VP16的化疗和鼻部放疗,但最终疾病未能控制而死亡。从最初诊断为HLH至最后死亡总共生存6个月。结论：本病例演示了从＂EBV-HLH进展至鼻区NK/T细胞淋巴瘤＂整个过程,探讨了EBV感染,HLH和NK/T淋巴瘤在发病机制上的相关性。结合文献,伴HLH的淋巴瘤预后差,而HLH出现在淋巴瘤之前较罕见,并且预后更差。目前尚缺乏标准的有效治疗方法。有报道对于高危患者尽早使用含VP16免疫化疗方案和异基因造血干细胞移植可改善患者生存,但仍需积累更多的成功治疗经验指导临床实践,以提高患者的生存率。

英文摘要：

Objective：To report a case of extranodal nasal NK/T cell lymphoma appeared as EB virus-associated hemophagocytic lymphoid histiocytosis （EBV HI.H）, and to review the relative literatures. Method：The clinical features of a rare case of nasal NK/T cell iymphoma with EBV-HLH as first symptoms were collocted,including disease history, physical examination, blood and bone marrow cytology, PET-CT imaging and histopathological findings. Resuit..A 27-year old female patient sufferd from fever,sore throat, cough and joint pain. Because of pro- gressive pancytopenia, fever, hepatosplenomegaly, low fibrinogen, high triglyceride and EBV-positive test, the patient was diagnosed as EBV-HLH. Then the patient received immunosuppressive therapy and her clinical features were improved. One month later, the patient suffered from herpes zoster and progressive obstruction of nose. Inferior turbinate biopsy and pathological analysis showed that tumor cells expressed CD56＋/- , TIA1＋ , GB＋ , PF＋ , CD3＋,CD43＋,L26 ,PAX5 ,CD21 ,CD30-,KP1 ,Ki 67＋ （60%） and EREB＋.Thus the patient was diagnosed as nasal-type NK/T cell lymphoma and received VP16 based chemotherapy plus radiotherapy. The patient finally died after 6 months. Conclusion：This case demonstrates the process from EBV-HLH to nasal NK/T cell lymphoma. It implies that the EBV infection, HLH and NK/T lymphoma are related. It is reported that lymphoma with HLH has worse prognosis than lymphoma without HLH. It is rare that HLH appeares before lymphoma and prognosis is the worst in this situation. There are no standard therapies for NK/T cell lymphoma with HLH. In some case reports early use of VP16 based regimens as well as allogeneie hematopoietic stem cell transplantation may be effective for high-risk patients. But it still need to be approved by further large scale clinical trials.