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母细胞性浆细胞样树突细胞肿瘤6例临床病理分析

Blastic plasmacytoid dendritic cell neoplasm:a clinicopathologic study of six cases
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摘要 目的探讨母细胞性浆细胞样树突细胞肿瘤(BPDCN)临床病理特征。方法收集6例BPDCN患者临床信息、病理资料;所有病例均行HE、免疫组化染色和EBV-EBER原位杂交。结果男性5例,女性1例,平均年龄45.2岁。均表现为皮肤肿瘤。组织学改变为真皮全层至皮下脂肪组织见肿瘤细胞结节状或弥散浸润,表皮未受累;细胞中等大小,较一致,核质比高,核圆形或不规则,染色质细腻,核分裂象多见。免疫表型6例肿瘤细胞CD4和CD123均(+),5例CD7和CD56(+),2例Td T弥漫(+),2例CD68(+),1例CD117灶性(+),Ki-67阳性范围30%~80%,CK、EMA、CD3、CD20、CD30、CD34、S-100、MPO(-);EBV-EBER均为(-)。结论 BPDCN是罕见的淋巴造血系统肿瘤,好发于皮肤,诊断需结合临床、形态学与免疫组化。 Objective To investigate the clinicopathological features of blastic plasmacytoid dendritic cell neoplasm(BPDCN).Methods Six BPDCN patients were investigated from 2011 to 2014 in the Department of Pathology,Guangzhou KINGMED Center for Clinical Laboratory Co.Ltd.HE staining,immunohistochemical staining and EBER in situ hybridization were performed in 6 cases.Results There were 5 males and 1 female,and the average age was 45.2(9 ~75).The lesions were manifested as skin masses.Histological examination showed a diffuse or nodular infiltrate composed of intermediate-sized cells in dermis sparing epidermis area with high nucleocytoplasmic ratio and circular nucleus and delicate chromatin,but no tumor cell in epidermis.All patients were positive for CD4 and CD123;5 cases were positive for CD7 and CD56;Ki-67 positive range was 30-80%.All patients were negative for CK,EMA,CD3,CD20,CD30,CD34,S-100,MPO and EBER.Conclusion BPDCN is a rare lymphatic hematopoietic system tumor,and it frequently implicates the skin.The diagnosis should be combined with clinical manifestation,morphology and immunohistochemistry.
出处 《诊断病理学杂志》 CSCD 2016年第12期909-911,共3页 Chinese Journal of Diagnostic Pathology
关键词 血液肿瘤 免疫表型分型 母细胞性浆细胞样树突细胞肿瘤 Hematologic neoplasms Immunophenotyping Blastic plasmacytoid denderitic cell neoplasm
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