母细胞性浆样树突细胞肿瘤13例临床病理学特征  

Blastic plasmacytoid dendritic cell neoplasm:A clinico-pathological retrospective analysis of thirteen cases

作　　者：农琳[1] 王微[1] 梁丽[1] 李东[1] 李鑫[1] 李挺[1] NONG Lin;WANG Wei;LIANG Li;LI Dong;LI Xin;LI Ting(Department of Pathology,Peking University First Hospital,Beijing 100034,China)

机构地区：[1]北京大学第一医院病理科,北京100034

出　　处：《北京大学学报（医学版）》2023年第2期308-314,共7页Journal of Peking University:Health Sciences

基　　金：北大医学青年科技创新培育基金(BMU2020PYB001);北京大学第一医院青年临床研究专项课题(2021CR21)。

摘　　要：目的:分析母细胞性浆样树突细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm,BPDCN)的临床病理学特征。方法:收集2013年1月至2022年3月北京大学第一医院确诊的BPDCN患者的病历资料共13例,回顾性分析患者的临床表现、组织病理学特征、免疫表型及其预后。结果:13例患者男性11例,女性2例,中位年龄62岁(5~78岁)。13例中单器官受累5例,均为皮肤受累;多器官受累8例(皮肤/脑/乳腺+骨髓受累3例,皮肤+骨髓+淋巴结受累3例,皮肤+骨髓+淋巴结+脾受累2例)。组织病理学分析以中等至大型幼稚母细胞一致性增生浸润为特征,浸润皮肤真皮全层,骨髓病变以弥漫性浸润为主,淋巴结受累时淋巴结结构完全破坏,脾累及者主要侵犯脾红髓。免疫组织化学染色显示,13例均不同程度阳性表达CD4、CD56、CD123(13/13),9例均表达TCL1(9/9);部分表达CD68(KP1)(8/13)、TdT(7/12),CD117(2/6),显示高Ki-67增殖指数(40%~80%);不表达CD20、CD3、CD34、MPO、CD30;EBER原位杂交阴性(0/9)。明确诊断后6例接受化疗,其中1例辅以放疗,2例接受后续骨髓移植;另有2例仅维持治疗;随访中位时间14个月(6~36个月),5例死于疾病进展(6~18个月),3例存活(7~36个月),5例失访。结论:BPDCN是罕见的恶性淋巴造血系肿瘤类型,侵袭性强,临床预后较差。诊断需结合临床特征、组织病理学、免疫组织化学表型,并注意与其他母细胞形态或CD4+CD56+淋巴造血系肿瘤相鉴别。Objective:To investigate the clinicopathological features of blastic plasmacytoid dendritic cell neoplasm(BPDCN).Methods:A total of 13 cases of BPDCN diagnosed in Peking University First Hospital from January 2013 to March 2022 were collected.The clinical features,histopathological characteristics,immunophenotypes and prognosis of the patients were analyzed retrospectively,and the related literatures was reviewed as well.Results:Among the 13 patients,11 were male and 2 were female,with a median age of 62 years(ranging from 5 to 78 years).Among them,single organ involvement occurred in 5 cases,all of which presented with skin lesions.Two or more organs were involved in other 8 cases(single organ with bone marrow involved in 3 cases;skin,bone marrow and lymph node involved simultaneously in 3 cases;skin,bone marrow,lymph node and spleen involved simultaneously in 2 cases).Histopathologically,it was characterized by the proliferation of medium to large atypical blastic cells,which infiltrated the whole thickness of dermis.When involved,the bone marrow lesions mainly appeared in a diffuse pattern,while the lymph node structure was usually destroyed,and the red pulp of the affected spleen was diffusely invaded.Immunohistochemical staining showed that all the 13 cases were positive for CD4,CD56,and CD123(13/13)in varying degrees.All the 9 cases expressed TCL1(9/9).Variable expression of CD68(KP1)(8/13),TdT(7/12),CD117(2/6),and high Ki-67 proliferation index(40%~80%)were showed.The neoplastic cells lacked expressions of CD20,CD3,MPO,CD34,or CD30;EBER in situ hybridization were negative(0/9).After definite diagnosis,6 cases received chemotherapy,among which 1 received adjuvant radiotherapy,and 2 received subsequent bone marrow transplantation.Another 2 cases only received maintenance treatment.The median follow-up time was 14 months(ranging from 6 to 36 months),5 patients died of the disease(6 to 18 months),3 patients survived(7 to 36 months up to now),and the remaining 5 patients lost follow-up.Conclusion:BPDCN is a rar

关 键 词：母细胞性浆样树突细胞肿瘤 临床病理学特征 CD56 CD123 TCL1 

分 类 号：R733[医药卫生—肿瘤]