结外鼻型NK/T细胞淋巴瘤30例诊疗分析

Analysis of diagnosis and treatment of 30 patients with extranodal NK/T-cell lymphoma, nasal type

目的 探讨结外鼻型NK/T细胞淋巴瘤的临床特征及预后影响因素.方法 对按照WHO淋巴瘤分类标准,经病理确诊的30例结外鼻型NK／T细胞淋巴瘤患者临床特点、长期生存率、预后因素进行分析.结果 30例结外鼻型NK/T细胞淋巴瘤患者,以男性患者居多,中位发病年龄45岁,年轻患者鼻外型较多见,且更易合并噬血细胞综合征.实验室检查发现白细胞、血红蛋白、血小板、丙氨酸氨基转移酶、乳酸脱氢酶、清蛋白常有异常,且在生存组与死亡组之间,血红蛋白下降、血清清蛋白下降程度的差异有统计学意义.死亡患者发病年龄相对较轻,鼻外型多见,更多伴B症状、噬血细胞综合征,国际预后指数（IPI）评分较高,骨髓累及发生率较高,临床分期更晚,实验室检查异常更多.Kaplan-Meier单因素分析显示发病年龄、是否鼻腔来源、有无B症状、有无骨髓累及、是否伴发噬血细胞综合征、IPI评分、临床分期、治疗方法,以及有无白细胞、血红蛋白、血小板、血清乳酸脱氢酶、清蛋白水平异常,有无肝功能损伤等因素,均可影响预后;采用Cox回归模型进行多因素预后分析,显示有无血清LDH升高及有无骨髓累及为影响预后的独立危险因素.结论 结外鼻型NK/T细胞淋巴瘤多见于中年男性,侵袭性高,病初临床表现多样.相比鼻腔NK/T细胞淋巴瘤患者,鼻外型患者发病年龄更轻,实验室异常更明显,更易伴发噬血细胞综合征,更易累及骨髓,预后更差.有无血清LDH升高及有无骨髓累及为影响预后的独立危险因素.

Objective To analyze the clinical characteristics of extranodal NK/T-cell lymphoma,nasal type,and its prognostic factors.Methods 30 patients with extranodal NK/T-cell lymphoma,nasal type were collected according to the WHO lymphoma classification criteria.The clinical characteristics,long-term survival rates and prognostic factors of the 30 patiets were analyzed.Results Extranodal NK/T-cell lymphoma,nasal type usually affected male patients,male to female ratio was approximately 4∶1,at a median age of 45 years old.The young patients was more common to merge the hemophagocytic syndrome.Extranodal NK/T-cell lymphoma might invole any anatomic site.Laboratory examination revealed white blood cell,hemoglobin,platelet,alanine aminotransferase,lactate dehydrogenase,albumin abnormalities.Between survival and death groups,hemoglobin abnormalities and albumin decreases might be meaningful.15 patients died at last,with ligter onset age,more common with extrnose type,and more with B symptoms,hemophagocytic syndrome,higher IPI scores,higher bone marrow involvingrate,later clinical stage,more abnormal laboratory tests.Kaplan-Meier univariate analysis found these factors can affect the prognosis,including the onset age,whether nasal sources,with or without B symptoms,IPI scores,associated hemophagocytic syndrome,with or without bone marrow involved,clinical stage,treatment methods,as well as white blood cells,hemoglobin,platelet levels,serum lactate dehydrogenase,albumin,with or without liver dysfunction and other factors.But the Cox regession multivariate prognostic analysis showed that serum lactate dehydrogenase levels and the presence of bone marrow involving were the independent prognostic risk factors.Conclusions Extranodal NK/T-cell lymphoma,nasal type is invasive,and more common in middle-aged men,it may have diverse clinical manifestations with early stage.Compared to nasal cavity NK/T cell lymphoma,extranasal type appearances in patients with lighter onset age,more pronounced laboratory abnormalities,more associated with hemophagocytic syndrome,more involved bone marrow,and with a worse prognosis.The serum lactate dehydrogenase levels and the presence of bone marrow involving are the independent prognostic risk factors.