摘要
目的对5例侵袭性NK细胞白血病(ANKL)的临床病理特征进行分析。方法回顾性分析2015年10月至2021年7月广西壮族自治区人民医院收治的5例ANKL患者的临床病理资料。采用免疫组化染色检测胞浆CD3、CD45RO、CD56、粒酶B(granzyme B)、T细胞胞浆内抗原-1(TIA-1)、CD4、CD5、CD8、CD20、髓过氧化物酶(MPO)、上皮钙黏附素(E-cadherin)、Ki-67的表达,采用原位杂交法检测爱泼斯坦-巴尔病毒(EBV)编码的小RNA(EBER)的表达。结果5例ANKL患者中男4例,女1例,年龄16~85岁。患者表现为高热(5/5),伴有肝脾肿大(3/5)、乳酸脱氢酶升高(5/5)、外周全血细胞减少(5/5)、凝血功能障碍(5/5)等。骨髓活检可见肿瘤细胞呈间质性或灶片状浸润,细胞体积中等大小,细胞多形性,有细胞凋亡及核分裂象。免疫组化染色示胞浆CD3、CD45RO、CD56、granzyme B、TIA-1阳性,CD4、CD5、CD8、CD20、MPO、E-cadherin阴性,Ki-67增殖指数约为80%。原位杂交检测结果显示肿瘤细胞EBER阳性。1例行化疗,生存期3个月;4例放弃治疗,生存期1~3个月。结论ANKL是一种罕见的系统性NK细胞肿瘤,与EBV感染密切相关,病程发展快速,需与其他成熟T细胞和NK细胞淋巴瘤/白血病鉴别诊断。
Objective To analyze the clinicopathological features of 5 cases of aggressive natural killer(NK)-cell leukemia(ANKL).Methods The clinicopathological data of 5 patients with ANKL who were admitted to the People's Hospital of Guangxi Zhuang Autonomous Region from October 2015 to July 2021 were retrospectively analyzed.Immunohistochemical staining was used to detect the expressions of cytoplasmic CD3,CD45RO,CD56 and granzyme B,and T-cell intracellular antigen-1(TIA-1),CD4,CD5,CD8,CD20,myeloperoxidase(MPO),E-cadherin and Ki-67.The expression of Epstein-Barr virus(EBV)-encoded small RNA(EBER)was detected by using in situ hybridization method.Results Among the 5 patients with ANKL,4 patients were male and 1 patient was female,aged 16-85 years.The patients presented with high fever(5/5),accompanied by hepatosplenomegaly(3/5),elevated lactate dehydrogenase(5/5),peripheral pancytopenia(5/5)and coagulation dysfunction(5/5).The results of bone marrow biopsy revealed that the tumor cells showed interstitial or patchy infiltration,with medium-sized cell volume and pleomorphic cells,and showed signs of apoptosis and mitosis in the tissue hematoxylin-eosin(HE)slices.The results of immunohistochemical staining showed that cytoplasmic CD3,CD45RO,CD56,granzyme B and TIA-1 were positive,while CD4,CD5,CD8,CD20,MPO and E-cadherin were negative.The Ki-67 proliferation index was approximately 80%.The detection results of in situ hybridization showed that the tumor cells were EBER positive.One patient underwent chemotherapy and survived for 3 months.Four patients gave up treatment and had a survival period of 1-3 months.Conclusion ANKL is a rare systemic NK cell tumor,which is closely related to EBV infection.The disease progresses rapidly and needs to be differentiated from other mature T-and NK-cell lymphomas/leukemia.
作者
刘霞
黄永塔
罗裕
郭文文
莫祥兰
LIU Xia;HUANG Yongta;LUO Yu;GUO Wenwen;MO Xianglan(Department of Pathology,the People's Hospital of Guangxi Zhuang Autonomous Region(Guangxi Academy of Medical Sciences),Nanning 530021,China;Department of Laboratory Medicine,the People's Hospital of Guangxi Zhuang Autonomous Region(Guangxi Academy of Medical Sciences),Nanning 530021,China)
出处
《中国临床新医学》
2025年第5期560-564,共5页
CHINESE JOURNAL OF NEW CLINICAL MEDICINE
基金
国家自然科学基金项目(编号:82160041)。
关键词
NK细胞
白血病
爱泼斯坦-巴尔病毒
病理学特征
鉴别诊断
Natural killer(NK)cells
Leukemia
Epstein-Barr virus(EBV)
Pathological feature
Differential diagnosis
作者简介
第一作者:刘霞,医学硕士,主治医师,研究方向:临床病理诊断。E-mail:a200550346@163.com;通信作者:黄永塔,医学硕士,副主任医师,研究方向:头颈部肿瘤、淋巴造血系统疾病临床病理诊断。E-mail:huangyongta@126.com。
