母细胞性浆细胞样树突状细胞肿瘤病理学观察  

Pathologic observation of blastic plasmacytoid dendritic cell neoplasm

作　　者：刘恩彬[1] 陈辉树[1] 张培红[1] 李占琦[1] 孙琦[1] 杨晴英[1] 方立环[1] 孙福军[1] 

机构地区：[1]中国医学科学院北京协和医学院血液病医院病理科,天津300020

出　　处：《诊断病理学杂志》2011年第4期257-260,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨母细胞性浆细胞样树突状细胞肿瘤(BPDCN)的组织病理学特点、诊断与鉴别诊断。方法对2例BPDCN患者的组织标本(1例为淋巴结、骨髓活检,另1例为皮肤、淋巴结、骨髓活检)进行HE染色及常规EliVision免疫组化观察与分析。结果 2例均为男性,年龄73岁和7岁。就诊时1例无症状、多发性皮肤病损,表面呈淡红色至紫红色的斑疹、丘疹、斑块、皮下结节;另1例无皮肤病损,以浅表淋巴结肿大就诊。组织学检查示1例侵犯淋巴结和骨髓;另1例侵犯皮肤、淋巴结和骨髓。镜下瘤细胞形态类似于淋巴母细胞或原始单核细胞。免疫组化示瘤细胞CD4、CD56、CD43和CD123(+),CD117、MPO、CD68、PAX5、CD20和CD3(-)。结论 BPDCN是一种罕见类型高度侵袭性血液肿瘤,除侵犯皮肤外还侵犯骨髓和淋巴结。CD4、CD56、CD43和CD123(+),不表达系别特异性标记,应注意与急性白血病、淋巴瘤区别。Objective To explore the histopathology,diagnosis and differential diagnosis of blastic plasmacytoid dendritic cell neoplasms（BPDCN）.Methods Tissue samples were obtained from 2 patients who were diagnosed as BPDCN,including skin and lymph node biopsies from 1 case and skin,lymph node and bone marrow biopsies from another.Histologic examination was performed on 4% buffered formalin fixed and hematoxylin-eosin（H-E）-stained sections with paraffin emdedded samples.Immunohistochemical study was conducted with routinely EliVision method.Results There were 2 males with age of 73 and 7.1 years,respectively.The patients presented with asymptomatic multiple skin lesions that were light red to purple macules,papules,plaques or subcutaneous nodules.1 patients presented with lymphadenopathy without skin lesions.Pathologic findings showed 1 had lymph node and bone marrow involvement;1 had skin,lymph node and bone marrow involvement.Microscopically,morphology of the tumor cells was similar to lymphoblasts or monoblasts.Immunohistochemical characterization of the tumors showed the expression of CD4,CD56,CD43 and CD123,whereas CD117,MPO,CD68,PAX5,CD20 and CD3 were negative.Conclusions BPDCN is a rare and highly aggressive hematopoietic neoplasm.Except for the skin,bone marrow and lymph node can also be involved.The neoplasmic cells express CD4,CD56,CD43,CD123 and lack the other lineage specific markers.It should be distinguished from acute leukaemia and lymphoma.

关 键 词：母细胞性浆细胞样树突状细胞肿瘤 病理学 免疫表型 骨髓活检 

分 类 号：R733.1[医药卫生—肿瘤]