母细胞性浆细胞样树突细胞肿瘤2例  

Blastic Plasmacytoid Dendritic Cell Neoplasm:Two Cases Report

作　　者：常莉[1] 王一宇[1] 牟妍[1] 金仙花[1] 李雪[1] 朱文静[1] 黄洋[1] 夏建新[1] 

机构地区：[1]吉林大学第二医院皮肤科,吉林长春130041

出　　处：《中国皮肤性病学杂志》2016年第3期281-283,297,共4页The Chinese Journal of Dermatovenereology

基　　金：吉林省科学技术厅基金(20130413015GH)

摘　　要：例1,患者女,85岁。全身泛发青紫色丘疹、结节2个月,无自觉症状。例2,患者女,33岁。左侧下腹部出现一紫红色结节3个月,质地硬,活动度差,边界尚清,压痛(+)。皮损组织病理示:两例组织学表现基本一致,表皮未见异常,真皮全层见中等大小、形态较一致的幼稚肿瘤细胞弥漫或灶状浸润,肿瘤组织与表皮之间形成无浸润带,肿瘤细胞核大,深染,不规则,核仁不明显。免疫病理学示:两例均表现CD4,CD56,CD123,CD31(+),CD3,CD20,CD117,MPO,EBER(-)。诊断:母细胞性浆细胞样树突细胞肿瘤。两例患者分别于确诊后1个月及8个月死亡。Two cases of blastic plasmacytoid dendritic cell neoplasm reported. Case 1 a 85-year-old woman presented with purple nodules on the whole body without self-conscious symptoms for 2 months. Case 2 a 33-year-old woman presented with a purplish red mass on the abdomen with hard texture and tenderness for 3months. The histopathologic examination of two cases showed diffuse infiltration of small / medium-sized immature cells in the dermis layer,with non-infiltration zone between dermis and epidermis. The morphology of tumor cells was atypical with larger indented hyperchromatic nuclei and scant cytoplasm. Under immunohistochemical staining,the neoplastic cells of two cases exhibited positive for CD4,CD56,CD123 and CD31,as well as negative for CD3,CD20,CD117,MPO and EBER. Based on all findings,a diagnosis of blastic plasmacytoid dendritic cell neoplasm was made. Case 1 died one month after the diagnosis and case 2 died eight months after that.

关 键 词：树突细胞 浆细胞 免疫表型 CD31 

分 类 号：R739.5[医药卫生—肿瘤]