结外NK/T细胞鼻型淋巴瘤5例临床分析

目的探讨结外NK/T细胞鼻型淋巴瘤临床特征、诊疗方案和预后。方法收集5例结外NK/T细胞淋巴瘤鼻型患者的临床资料,分析其临床表现、辅助检查、治疗情况及追踪结局,结合相关文献进行总结。结果 5例患者以鼻塞、鼻出血、咽痛、腭部溃疡为主要临床表现。最终靠病理活检及免疫组化明确诊断。根据Ann Arbor分期:ⅡE期1例,ⅢE期2例,ⅣE期2例。有B症状4例。5例患者预后评估1例中高危,4例高危。2例接受含有左旋门冬酰胺酶的方案化疗及联合放疗的患者1例完全缓解,1例部分缓解,定期门诊随访;3例放弃治疗的患者均死亡,自然病程小于6个月。结论结外NK/T细胞淋巴瘤鼻型属T细胞侵袭性淋巴瘤,其发病与EB病毒关系密切,恶性程度高,目前治疗方法主要有放疗、化疗及骨髓移植,靶向治疗可作为今后的研究方向。

Objective To discuss the clinical characteristic of the nasal type extranodal NK/T cell lymphoma（ENK-TCL） as well as the therapeutic regimen and prognosis. Methods The clinical information of the 5 patients confirmed ENK-TCL were collected, the clinical manifestation, auxiliary examinations, treatments were analyzed and tracked the end. Results The main clinical features of the 5 patients were rhinobyon, epistaxis, sore throat and jaws ulcer. They were fanally diagnosed by pathologic biopsy and immunohistochemistry. Ann Arbor stage II E 1 case, IIIE 2 cases and WE 2 cases. B symptoms 4 cases. One case was at high risk of prognostic evaluation,4 cases were high risk.2 patients received chemotherapy contains L-asparaginasum and radiotherapy, of whom 1 case received complete remission（CR） and 1 case partial remission（PR）, and outpatient service follow-up regularly,3 cases abandoning theropy died within 6 months. Conclusion ENK-TCL is belong to T cell aggressive lymphoma and related to Epstein-Barr（EB） virus. Malignant degree is high. The main treatments are radiotherapy, chemotherapy and bone marrow transplantation, and the targeted therapy could be the future research area.