BPDCN:最先进的。

IF 2.9 3区 教育学 Q1 EDUCATION, SCIENTIFIC DISCIPLINES
Naveen Pemmaraju
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引用次数: 0

摘要

母细胞浆细胞样树突状细胞肿瘤(BPDCN)在血液系统恶性肿瘤的万神殿中作为其独特的实体出现,是由于人们对其独特的多器官临床表现和特征性皮肤病变的认识日益加深。BPDCN的发生通常表现为多室性皮肤病变,骨髓和/或血液受累,淋巴结浸润,并倾向于髓外器官受累,包括最显著的中枢神经系统(CNS)/脑脊液阳性。在该领域的早期研究中,中位年龄≥70岁,男性占比高达5:1,现代最显著的发展是对新兴的BPDCN重要群体的认识,如女性、儿科和青少年/青壮年患者;CNS + BPDCN患者;世界范围内确诊的病例也越来越多。这些趋势与世界各地血液学/肿瘤学、血液病理学、皮肤病学和皮肤病理学团队增加的教育和研究努力、更大的国际合作以及显著改进的诊断工具和临床方法相一致。现在,自首个专门用于BPDCN的商业批准靶向药物——cd123靶向药物tagraxofusp以来,已有5年多的时间,特别是在BPDCN患者的一线环境中,已经证明了改善。该领域充满了希望,因为它经历了许多进步,包括更大的分子表征,扩展了CD123以外治疗潜在靶点的识别,联合治疗的出现,干细胞移植参数的改善,以及专门针对BPDCN患者的新型临床试验。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
BPDCN: state of the art.

The emergence of blastic plasmacytoid dendritic cell neoplasm (BPDCN) as its own distinct entity within the pantheon of hematologic malignancies is due to the growing understanding of its unique multiorgan clinical presentation and characteristic skin lesions. The occurrence of BPDCN is generally heralded by a multicompartmental presentation of violaceous cutaneous lesions, involvement by bone marrow and/or blood, lymph node invasion, and an inclination toward extramedullary organ involvement, including, most remarkably, central nervous system (CNS)/cerebrospinal fluid positivity. With a median age historically of ≥ 70 years and up to 5:1 male predominance in most of the field's earlier studies, the most notable development in the modern era is the recognition of emerging important groups with BPDCN, such as female, pediatric, and adolescent/young adult patients; CNS + BPDCN patients; and an increasing number of cases being diagnosed worldwide. These trends are in line with the increased educational and research efforts, greater international collaboration, and markedly improved diagnostic tools and clinical approaches among hematology/oncology, hematopathology, dermatology, and dermatopathology teams around the world. Now, with over 5 years since the first commercially approved targeted agent specifically dedicated for BPDCN, the CD123-targeted agent tagraxofusp, improvements have been demonstrated particularly in the frontline setting for patients with BPDCN. The field is abundant with hope, as it has experienced advancements including greater molecular characterization, expanded identification of potential targets for therapy beyond CD123, advent of combination therapies, improving parameters for stem cell transplantation, and novel clinical trials specifically available for patients with BPDCN.

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来源期刊
Hematology. American Society of Hematology. Education Program
Hematology. American Society of Hematology. Education Program EDUCATION, SCIENTIFIC DISCIPLINES-HEMATOLOGY
CiteScore
4.70
自引率
3.30%
发文量
0
期刊介绍: Hematology, the ASH Education Program, is published annually by the American Society of Hematology (ASH) in one volume per year.
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