母细胞性浆细胞样树突细胞肿瘤一例  

Blastic plasmacytoid dendritic cell neoplasm： a case report

作　　者：王亚哲[1] 张嵩[2] 郑姝颖[1] 江滨[1] 刘艳荣[1] 陈珊珊[1] 路瑾[1] 

机构地区：[1]北京大学血液病研究所、北京大学人民医院,100044 [2]河北联合大学附属医院血液科

出　　处：《中华皮肤科杂志》2013年第7期457-461,共5页Chinese Journal of Dermatology

摘　　要：患者女，19岁，左下肢暗红色斑丘疹1年余，右腹股沟可触及一樱桃大小淋巴结。皮肤肿物组织病理检查：皮肤正常结构消失，可见淋巴样肿瘤细胞弥漫浸润，细胞中等大小，核仁不清楚；免疫组化：CD4（＋＋）、CD56（＋＋）、CD123（＋＋＋），TdT部分阳性。骨髓穿刺行流式细胞检查：异常细胞占59．9％，表达CD123st（IL-3Ra）、HLA—DRst、CD56、CD304（BDCA-4）、CD7、CD11b、CD33、CD36、CXCR4和CD13dim，部分细胞表达CD4和CD117。透射电镜观察骨髓细胞：可见大量形态大小均一的淋巴样细胞，细胞表面可见粗大突起，胞核胞质比例高，胞核圆形居中，有呈脑回样深切迹，异染色质块状边集，核仁多见，少量胞质，其中可见内质网、线粒体等细胞器，内质网均呈浆细胞样同心圆状排列。骨髓细胞表现兼具树突细胞及浆细胞特征。诊断：母细胞性浆细胞样树突细胞肿瘤。A 19-year-old female presented with a dark erythematous maculopapule measuring 2 cm in diameter on the left lower limb for more than one year. Physical examination revealed an enlarged cherry-like lymph node in the right inguinal region. Histopathology of the lesion revealed that the normal structure of skin disappeared absolutely, and there was a diffuse infiltrate of medium-sized lymphoid tumor ceils with unclear nucleoli. Immunohistoehemieally, the tumor ceils strongly expressed CD4, CD56 and CD123, and partially expressed terminal deoxynucleotidyl transferase （TdT）. Flow eytometric analysis of bone marrow aspirates showed that abnormal cells amounted to 59.9%, and were positive for CD123st （strong）, human leucocyte antigen-DRst （strong）, CD56, CD304 （blood dendritic cell antigen-4）, CD7, CDllb, CD33, CD36 and chemokine （C-X-C motif） receptor 4, CD13 （dim）, CD4 （partial） and CDll7 （partial）. Transmission electron microscopy of bone marrow cells revealed that there were massive uniformly sized lymphoid ceils with thick processes on cell surfaces, a high nucleus/cytoplasm ratio, centrally located round nuclei occasionally with deep gyrus-like notches, clumped marginated heteroehromatin and multiple nucleoli. Moreover, a small amount of cytoplasm was observed in the lymphoid cells with the presence of mitochondria and endoplasmic reticulum arranged in concentric circles, which were characteristic of plasma cells. The features of both dendritic cells and plasma cells were found in the tumor cells through transmission electron microscopy. Based on the above findings, a diagnosis of blastic plasmacytoid dendritic cell neoplasm （BPDCN） was made.

关 键 词：白血病 髓样 树突细胞 浆细胞 

分 类 号：R73[医药卫生—肿瘤]