国际肿瘤学杂志

国际肿瘤学杂志 ›› 2019, Vol. 46 ›› Issue (10): 577-580.doi: 10.3760/cma.j.issn.1673-422X.2019.10.001

• 卫生健康事业发展70年巡礼 •    下一篇

母细胞性浆细胞样树突状细胞肿瘤治疗新进展

杨婧诗1,邹立群2   

  1. 1四川大学华西临床医学院,成都610041； 2四川大学华西医院肿瘤一科，成都610041

  • 出版日期:2019-10-08 发布日期:2019-12-20
  • 通讯作者: 通信作者:邹立群，Email: hxlcyxy@163.com E-mail:hxlcyxy@163.com

New progression in the therapy of blastic plasmacytoid dendritic cell neoplasms

Yang Jingshi1, Zou Liqun2   

  1. 1West China School of Medicine, Sichuan University, Chengdu 610041, China; 2First Department of Oncology, West China Hospital, Sichuan University, Chengdu 610041, China

  • Online:2019-10-08 Published:2019-12-20
  • Contact: Zou Liqun, Email: hxlcyxy@163.com E-mail:hxlcyxy@163.com

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摘要：

母细胞性浆细胞样树突状细胞肿瘤（BPDCN）是一种罕见的具有高度侵袭性的血液系统恶性肿瘤。因其发病率低，目前尚无标准治疗方案。年轻患者常采用高强度化疗联合造血干细胞移植。无法行造血干细胞移植的老年患者采用低强度化疗。2018年12月，美国食品药物监督管理局批准新型靶向药tagraxofusp专门用于初治或复发BPDCN患者（年龄≥2岁）的治疗；venetoclax、daratumumab等靶向药物均在治疗BPDCN中显示出一定的疗效；PD-1/PD-L1抑制剂以及抗CD123嵌合抗原受体T细胞免疫治疗的疗效仍需进一步研究。

关键词: 血液肿瘤, 治疗, 母细胞性浆细胞样树突状细胞肿瘤

Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a kind of rare and highly invasive hematological malignancy. Because of its low incidence, there is still no consensus on its standard treatment. For young patients, high intensity chemotherapy combined with hematopoietic stem cell transplantation is often used. Elderly patients who cannot accept hematopoietic stem cell transplantation receive low intensity chemotherapy. In December 2018, tagraxofusp, a new targeted drug, was approved by the U.S. Food and Drug Administration specially for treatment-naive and previously-treated BPDCN patients (age≥2 years old). Some targeted drugs, such as venetoclax and daratumumab, have certain effects on the treatment of BPDCN. The efficacies of PD-1/PDL-1 inhibitors and anti CD123 chimeric antigen receptor T cell immunotherapy need further researches.

Key words: Hematological neoplasms, Therapy, Blastic plasmacytoid dendritic cell neoplasms