母细胞性浆细胞样树突细胞肿瘤的临床及病理特点  

Clinical and Pathological Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm

作　　者：董菲[1] 张朕豪 高帆 甄敬飞 郑东 赵伟[1] 李敏[2] 景红梅[1] DONG Fei;ZHANG Zhen-Hao;GAO Fan;ZHEN Jing-Fei;ZHENG Dong;ZHAO Wei;LI Min;JING Hong-Mei(Department of Hematology,Peking University Third Hospital,Beijing 100191,China;Department of Pathology,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学第三医院血液科,北京100191 [2]北京大学第三医院病理科,北京100191

出　　处：《中国实验血液学杂志》2020年第2期518-523,共6页Journal of Experimental Hematology

摘　　要：目的:探讨母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床表现、病理特点、治疗方案及预后。方法:回顾性分析经病理证实的5例BPDCN患者的临床病理特征、诊断治疗及预后。结果:本组5例患者中4例存在皮肤损害,5例患者均有骨髓累及。免疫组织化学检测显示,5例患者均表达CD56,4例患者表达CD4和CD123,3例患者表达CD43。经初始方案治疗后3例达完全缓解,1例达到部分缓解1个月后疾病进展,1例未缓解。2例年轻患者均进行了同胞全相合allo-PBSCT,这2例患者获得了长期生存。结论:BPDCN常见皮肤损害、骨髓受累,可表达CD56、CD3、CD123及CD43。对于合适的患者在CR1期积极进行异基因外周血干细胞移植,可能增加其获得长期生存的机会。Objective:To explore the clinical manifestation,pathological features and treatment regimen of blastic plasmacytoid dendritic cell neoplasm(BPDCN).Methods:The clinical and pathologic features,diagnosis,treatment and prognosis of 5 BPDCN patients were analyzed retrospectively.Results:5 patients had skin lesions,and bone marrow involvement.The immunohistochemical staining showed that the CD56 expression was observed in 5 patients,CD4 and CD123 in 4 patients,and CD43 expressed in 3 patients.After initial treatment,3 patients achieved complete remission and 1 patient did not.One patient showed disease progression after partial remission for 1 month.Two young patients underwent sibling allo-PBHSCT,and then achieved long-term survival.Conclusion:BPDCN often presents skin lesions as the first symptom,moreover,soft tissues,lymph nodes and bone marrow were involved,which suggested the disease progresses.There is currently no uniform treatment protocol for this disease.Active allogeneic peripheral blood hematopoietic stem cell transplantation in the CR1 phase for appropriate patients may increase their chances for long-term survival.

关 键 词：母细胞性浆细胞样树突细胞肿瘤 临床病理特点 化疗 

分 类 号：R733.1[医药卫生—肿瘤] R733.71文献标识码A[医药卫生—临床医学]