母细胞性浆细胞样树状突细胞肿瘤临床病理学观察  

Clinical Pathological Observation of Blastic Plasmacytoid Dendritic Cell

作　　者：王瑞国[1] 罗晓青[1] 潘华雄[2] 

机构地区：[1]湖北医药学院附属襄阳医院病理科,湖北襄阳441000 [2]华中科技大学同济医学院附属协和医院病理科,湖北武汉430022

出　　处：《湖北医药学院学报》2013年第4期306-310,363,共6页Journal of Hubei University of Medicine

摘　　要：目的:研究母细胞性浆细胞样树状突细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm,BPDCN)的临床病理特征和免疫表型,探讨其诊断依据及鉴别诊断要点。方法:收集本院1例皮肤BPDCN患者的临床病理学及免疫组化特征,并复习文献。结果:BPDCN好发于老年男性,常以无症状性孤立或多发肿块为首发症状,可多部位受累,偏嗜皮肤,组织学形态表现为中等大小肿瘤细胞弥漫浸润真皮及皮下组织,瘤细胞胞质少,核不规则,染色质细,核分裂多见,无坏死及嗜表皮性。免疫组化:肿瘤细胞CD123、CD56、CD43阳性,少数细胞CD4、CD7和TdT阳性,B系、T系及髓、单核系标记均阴性。结论:BPDCN是罕见的淋巴造血系统高度侵袭性肿瘤,结合瘤细胞形态、生长方式及免疫表型有助于诊断,同时应注意与其他皮肤原发或继发淋巴造血系统肿瘤相鉴别。Objective To study the clinical pathological features and immunohistochemical phenotype of blastic plasmacytoid dendritic cell neoplasm(BPDCN),to explore the criteria of differential diagnosis.Methods The clinical pathological and immunohistochemical characteristics of one patient with skin BPDCN were observed,and the related literatures were reviewed.Results BPDCN occurs mainly in elderly males and its initial symptoms are the asymptomatic isolated or multiple lumps,which may involve multiple sites,especially the skin.The histological morphology manifeste as a homogeneous medium sized tumor cells diffused infiltrated into dermis and subcutaneous tissue.The cytoplasm of tumor cells was few and with thin lightstaining,the nuclei were irregularly folded and convoluted,the mitotic were common,there was no necrosis and it was epidermotropic.The results of immunohistochemistry showed that CD123,CD56,CD43 were positive in tumor cells,CD4,CD7 and TdT were positive in few of them,B-cell line,T-cell line and monocyte-line age markers were all negative.Conclusion BPDCN is a rare highly aggressive neoplasm of hematopoietic and lymphoid tissues,the combination of tumor cell morphology,growth pattern and immunophenotype could be helpful for diagnosis.Meanwhile,BPDCN should be differentiated from other primary or secondary skin lymphoid hematopoietic tumors.

关 键 词：母细胞性 浆细胞样树状突细胞 免疫组化 鉴别诊断 

分 类 号：R739.5[医药卫生—肿瘤]