天津医药

天津医药 ›› 2018, Vol. 46 ›› Issue (8): 828-831.doi: 10.11958/20180264

• 专题 临床研究 • 上一篇    下一篇

母细胞性浆细胞样树突状细胞瘤一例报告

牛海玥， 曹秋颖， 齐薇薇， 王一浩， 江汇涓， 王化泉， 邵宗鸿   

  1. 天津医科大学总医院血液科 （邮编300052）
  • 收稿日期:2018-02-22 修回日期:2018-06-27 出版日期:2018-08-15 发布日期:2018-08-23
  • 通讯作者: 牛海玥 E-mail:369210478@qq.com
  • 基金资助:
    CD4+CXCR5+细胞在骨髓增生异常综合征发病机制中作用的研究;重型再生障碍性贫血中免疫激活抗原的研究;CXCR5+CD8+T细胞在重型再生障碍性贫血免疫发病机制中作用的研究;MDSC介导的免疫抑制在骨髓增生异常综合征中作用的研究

A case of blastic plasmacytoid dendritic cell neoplasm

NIU Hai-yue, CAO Qiu-ying, QI Wei-wei, WANG Yi-hao, JIANG Hui-juan, WANG Hua-quan, SHAO Zong-hong   

  1. Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, China
  • Received:2018-02-22 Revised:2018-06-27 Published:2018-08-15 Online:2018-08-23
  • Contact: Hai-Yue NIU E-mail:369210478@qq.com

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摘要： 母细胞性浆细胞样树突状细胞瘤 （BPDCN） 是一种罕见的造血系统肿瘤， 恶性程度高， 进展迅速， 对化疗敏感， 但易复发耐药， 生存时间短。现报告我科收治的1例BPDCN患者， 以皮损为首发表现， 经皮肤活检确诊。初期对淋巴细胞肿瘤方案敏感但很快复发， 累及肺、 纵隔， 后予髓系肿瘤方案， 效果不佳， 最终死亡， 总生存期17个月。

关键词: 血液肿瘤, 白血病, 髓样, 急性, 母细胞性浆细胞样树突状细胞瘤, 诊断, 治疗

Abstract:  Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic neoplasm with high malignancy and rapid progression. BPDCN is sensitive to chemotherapy, but it is prone to relapse and drug resistance, and the survival time is short. We report a case of BPDCN hospitalized in our department. In this case, the skin lesions were the first manifestation. The diagnosis of BPDCN was confirmed by the skin biopsy. Initially, this patient was sensitive to the lymphoma chemotherapy but the disease relapsed quickly, spreading to the lungs and mediastinum. Then, we treated the patient with myeloid regimen, which was no efficacy. The patient eventually died with an overall survival of 17 months.

Key words: hematologic neoplasms, leukemia, myeloid, acute, blastic plasmacytoid dendritic cell neoplasm, diagnosis, treatment