陈晓君;刘彦权;胡晓梅;黄素蓉;沈建箴;
CHEN Xiaojun;LIU Yanquan;HU Xiaomei;HUANG Surong;SHEN Jianzhen;Department of Hematology, Fujian Institute of Hematology, National Key Laboratory of Hematology, Fujian Medical University Union Hospital;Department of Oncology, The First Affiliated Hospital of Gannan Medical University;Department of Pathology, Fujian Medical University Union Hospital;

• 1:福建医科大学附属协和医院血液科福建省血液病研究所国家血液病学重点实验室
• 2:赣南医学院第一附属医院肿瘤科
• 3:福建医科大学附属协和医院病理科

摘要(Abstract)：

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。
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.

关键词(KeyWords)： 母细胞性浆细胞样树突状细胞肿瘤;CD56阳性;阿扎胞苷;吉西他滨;奥沙利铂;抗肿瘤联合化疗方案
blastic plasmacytoid dendritic cell neoplasm;CD56 positive;azacitidine;gemcitabine;oxaliplatin;antineoplastic combined chemotherapy protocols

Abstract:

Keywords:

基金项目(Foundation): 国家自然科学基金（81300428）;; 福建省血液医学中心建设项目（闽卫医政【2017】171号）;; 国家及福建省重点专科临床重点专科建设项目（闽卫医函【2011】1018号、闽卫科教【2012】149号）~~

作者(Authors): 陈晓君;刘彦权;胡晓梅;黄素蓉;沈建箴;
CHEN Xiaojun;LIU Yanquan;HU Xiaomei;HUANG Surong;SHEN Jianzhen;Department of Hematology, Fujian Institute of Hematology, National Key Laboratory of Hematology, Fujian Medical University Union Hospital;Department of Oncology, The First Affiliated Hospital of Gannan Medical University;Department of Pathology, Fujian Medical University Union Hospital;

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