侵袭性NK细胞白血病的临床特点分析  

Clinical Characteristics of Aggressive NK-Cell Leukemia

作　　者：廖怡 何合胜 魏元凤 沈大鹏 季欣悦 黄辰 黄君 靳小可 黄东平 LIAO Yi;HE He-Sheng;WEI Yuan-Feng;SHEN Da-Peng;JI Xin-Yue;HUANG Chen;HUANG Jun;JIN Xiao-Ke;HUANG Dong-Ping(Department of Hematology,The First Affiliated Hospital of Wannan Medical College,Yijishan Hospital,Wuhu 241000,Anhui Province,China)

机构地区：[1]皖南医学院第一附属医院,弋矶山医院血液科,安徽芜湖241000

出　　处：《中国实验血液学杂志》2023年第4期1026-1031,共6页Journal of Experimental Hematology

基　　金：安徽省重点研究与开发计划项目(201904a07020036)。

摘　　要：目的:探讨总结侵袭性NK细胞白血病(ANKL)的临床特点和治疗方案,为该病的临床诊断、治疗提供新的认识与见解。方法:回顾性分析2014年3月至2021年7月皖南医学院第一附属医院收治的7例ANKL患者的临床资料,分析其临床特点、实验室和影像学结果及治疗与转归。结果:7例患者中5例男性,2例女性,中位年龄47(33-69)岁。7例患者骨髓细胞形态学均可见类似大颗粒淋巴细胞;5例免疫分型提示异常NK细胞。截至随访结束,6例死亡,1例存活,中位生存时间76.9(4-347)天。结论:ANKL在临床上罕见,病程短,预后很差,合并噬血细胞综合征时预后更差,目前治疗上没有统一的方法,部分患者使用PD-1抑制剂可能延长生存期。Objective:To explore and summarize the clinical characteristics and treatment of aggressive NK-cell leukemia(ANKL),and provide new insights for clinical diagnosis and treatment of this disease.Methods:The clinical data of 7 patients with ANKL admitted to the First Affiliated Hospital of Wannan Medical College from March 2014 to July 2021 were retrospectively analyzed,and their clinical characteristics,laboratory and imaging results,treatment and outcomes were analyzed.Results:Among the 7 patients,5 were males and 2 were females,with a median age of 47(33-69)years old.The morphology of bone marrow cells in 7 patients showed similar large granular lymphocytes.Immunophenotyping revealed abnormal NK cells in 5 cases.By the end of follow-up,6 cases died and 1 case survived,with a median survival time of 76.9(4-347)days.Conclusion:ANKL is a rare disease with short course and poor prognosis.If combined with hemophagocytic syndrome(HPS),the prognosis is even worse.There is no unified treatment method at present,and the use of PD-1 inhibitors may prolong the survival in some patients.

关 键 词：侵袭性NK细胞白血病 流式细胞学 程序性死亡蛋白-1(PD-1) 

分 类 号：R733.7[医药卫生—肿瘤]