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Regimen of Azacitidine Combined with GemOx in the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report
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摘要: 母细胞性浆细胞样树突状细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm，BPDCN)是一种来源于浆细胞样树突状细胞前体细胞、高度恶性的血液系统肿瘤。原发于鼻腔的BPDCN临床极为罕见，易误诊、漏诊，目前关于BPDCN的治疗，国内外尚无标准方案。本文报道1例原发于鼻腔的BPDCN患者，在采用阿扎胞苷联合GemOx方案治疗后病情得到显著控制、临床症状改善明显。笔者通过对该患者的临床诊疗过程进行分析与探讨，以期提高临床医师对罕见BPDCN的认识。Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly malignant hematological tumor derived from the precursor cells of plasmacytoid dendritic cells. BPDCN that primarily originates in the nasal cavity is clinically rare and easily misdiagnosed or missed. At present, there is no standard treatment for BPDCN in China and abroad. This article reported a female patient with BPDCN primarily in the nasal cavity. The regimen of Azacitidine combined with GemOx had a good clinical effect on this patient and significantly controlled the condition and improved the clinical symptoms. This article analyzed and discussed the clinical diagnosis and treatment of this patient, aiming to improve clinicians' understanding of rare BPDCN.作者贡献：陈晓君负责收集资料、查阅文献和论文撰写；刘彦权负责论文修改和校对；胡晓梅、黄素蓉负责患者图片收集和整理；沈建箴负责论文审核。利益冲突：所有作者均声明不存在利益冲突注：本文发表已获得患者知情同意
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图 3 患者化疗前骨髓免疫组化(多聚螯合物酶法En Vision, ×200)及常规病理染色(HE，×200)结果
A.CD4(+++)；B.CD56(+++)；C.CD123(弱++)；D.常规病理染色
图 4 鼻腔肿物活检免疫组化染色结果(多聚螯合物酶法En Vision，×200)及常规病理染色(HE，×200)结果
A.CD4(+)；B.CD56(+)；C.CD123(+)；D.Ki-67指数(50%+)
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