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阿扎胞苷联合GemOx方案治疗母细胞性浆细胞样树突状细胞肿瘤一例

陈晓君 刘彦权 胡晓梅 黄素蓉 沈建箴

陈晓君, 刘彦权, 胡晓梅, 黄素蓉, 沈建箴. 阿扎胞苷联合GemOx方案治疗母细胞性浆细胞样树突状细胞肿瘤一例[J]. 协和医学杂志, 2022, 13(1): 124-129. doi: 10.12290/xhyxzz.2021-0465
引用本文: 陈晓君, 刘彦权, 胡晓梅, 黄素蓉, 沈建箴. 阿扎胞苷联合GemOx方案治疗母细胞性浆细胞样树突状细胞肿瘤一例[J]. 协和医学杂志, 2022, 13(1): 124-129. doi: 10.12290/xhyxzz.2021-0465
CHEN Xiaojun, LIU Yanquan, HU Xiaomei, HUANG Surong, SHEN Jianzhen. Regimen of Azacitidine Combined with GemOx in the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report[J]. Medical Journal of Peking Union Medical College Hospital, 2022, 13(1): 124-129. doi: 10.12290/xhyxzz.2021-0465
Citation: CHEN Xiaojun, LIU Yanquan, HU Xiaomei, HUANG Surong, SHEN Jianzhen. Regimen of Azacitidine Combined with GemOx in the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report[J]. Medical Journal of Peking Union Medical College Hospital, 2022, 13(1): 124-129. doi: 10.12290/xhyxzz.2021-0465

阿扎胞苷联合GemOx方案治疗母细胞性浆细胞样树突状细胞肿瘤一例

doi: 10.12290/xhyxzz.2021-0465
基金项目: 

国家自然科学基金 81300428

福建省血液医学中心建设项目 Fujian Mdical Administration[2017] No. 171

国家及福建省重点专科临床重点专科建设项目 Fujian Mdical Letter[2011] No. 1018

国家及福建省重点专科临床重点专科建设项目 Fujian Health Science and Education[2012] No. 149

详细信息
    通讯作者:

    沈建箴, E-mail: doctorsjz@163.com

  • 中图分类号: R552; R733

Regimen of Azacitidine Combined with GemOx in the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Funds: 

National Natural Science Foundation of China 81300428

Fujian Blood Medical Center Construction Project Fujian Mdical Administration[2017] No. 171

National and Fujian Provincial Key Clinical Specialty Construction Project Fujian Mdical Letter[2011] No. 1018

National and Fujian Provincial Key Clinical Specialty Construction Project Fujian Health Science and Education[2012] No. 149

More Information
  • 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。
    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
  • 图  1  患者初入院时全身多发皮损

    A.颜面部;B.躯干

    图  2  患者化疗前骨髓涂片及细胞化学染色结果(×1000)

    A.骨髓涂片;B.POX染色(-);C.PAS染色(+); D.AS-DCE染色(-)

    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%+)

    图  5  患者阿扎胞苷联合GemOx方案化疗1个疗程(A、B)及4个疗程(C、D)后,面部肿块逐渐变平、缩小,仅残留少许色素沉着,骨髓涂片逐渐接近正常

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出版历程
  • 收稿日期:  2021-06-12
  • 录用日期:  2021-07-29
  • 网络出版日期:  2021-09-15
  • 刊出日期:  2022-01-30

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