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浆细胞样树突状细胞肿瘤

英文原题:Plasmacytoid dendritic cell neoplasms.

查看英文原题

Plasmacytoid dendritic cell neoplasms.

PubMed 2023/04/30(内容时间) Blood Res Q2 · IF 3.7(JCR 2025)

研究概要

浆细胞样树突状细胞 (pDC) 是产生 I 型干扰素、调节免疫应答的细胞。

中文摘要

浆细胞样树突细胞(pDC)可产生I型干扰素并调节免疫反应。pDC肿瘤有两类:与髓系肿瘤相关的成熟pDC增殖(MPDCP),以及母细胞性pDC肿瘤(BPDCN)。MPDCP是成熟pDC克隆性扩增,主要与慢性粒单核细胞白血病相关;BPDCN则是侵袭性髓系恶性肿瘤,可累及皮肤、骨髓、淋巴器官和CNS。皮肤病灶表现多样,可从孤立棕色或紫色斑块到全身播散性皮损。BPDCN典型免疫表型为CD4、CD56、CD123及pDC标志物(如TCL-1、TCF4、CD303、CD304)阳性。历史上BPDCN按急性白血病方案治疗,部分患者进行异基因造血细胞移植。分子生物学及遗传学进展推动靶向药开发,包括靶向CD123的重组融合蛋白tagraxofusp、抗CD123 CAR-T、XmAb14045和IMGN632。本文全面综述pDC肿瘤。

展开英文摘要原文

Plasmacytoid dendritic cells (pDCs) are type I interferon-producing cells that modulate immune responses. There are two types of pDC neoplasms: 1) mature pDC proliferation (MPDCP) associated with myeloid neoplasm and 2) blastic pDC neoplasm (BPDCN). MPDCP is a clonal expansion of mature pDCs that is predominantly associated with chronic myelomonocytic leukemia. In contrast, BPDCN is a clinically aggressive myeloid malignancy involving the skin, bone marrow, lymphatic organs, and central nervous system. There are various types of skin lesions, ranging from solitary brown or violaceous to disseminated cutaneous lesions, which often spread throughout the body. The expression of CD4, CD56, CD123, and pDC markers (TCL-1, TCF4, CD303, and CD304, etc.) are typical immunophenotype of BPDCN. Historically, BPDCN treatment has been based on acute leukemia regimens and allogeneic hematopoietic cell transplantation in selected patients. Recent advances in molecular biology and genetics have led to the development of targeted agents, such as tagraxofusp (a recombinant fusion protein targeting CD123), anti-CD123 CAR-T cells, XmAb14045, and IMGN632. Lastly, this review provides a comprehensive overview of pDC neoplasms.

论文信息

作者
Lee YJ、Kim Y、Park SH、Jo JC
单位
Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.South Korea
文献类型
综述
期刊
Blood research2023 Apr 30
原文标识
PubMed 37105563 · DOI 10.5045/br.2023.2023052