大疱性浆细胞性树突状细胞肿瘤 (BPDCN):临床特征和组织病理学及治疗概述

IF 1.1 Q4 HEMATOLOGY Hematology Reports Pub Date : 2023-12-08 DOI:10.3390/hematolrep15040070
Gerardo Cazzato, Marialessandra Capuzzolo, E. Bellitti, Giovanni De Biasi, A. Colagrande, Katia Mangialardi, F. Gaudio, G. Ingravallo
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引用次数: 0

摘要

母浆细胞样树突状细胞肿瘤(BPDCNs)是一种罕见的、高度侵袭性的血液恶性肿瘤,主要累及皮肤、骨髓、淋巴结甚至结外部位。文献中病例的罕见性和相对较差的描述使得有必要进行回顾和进一步的研究,不仅在组织病理学上,而且在分子领域深入调查这一实体。2023年8 - 9月,我们检索了MEDLINE、PubMed和Scopus,检索了近25年来发表的英文随机对照试验(rct)、叙事性和系统性综述、荟萃分析、观察性研究(纵向或回顾性)和病例系列,检索关键词为BPDCN、PDCs、Blastic NK细胞淋巴瘤、颗粒性CD4+ NK白血病/淋巴瘤、颗粒性CD4+ CD56+血皮病肿瘤/肿瘤。尽管近年来在该病的诊断和生物学认识方面取得了进展,但直到2018年,对其治疗还没有明确的共识,所使用的主要治疗方案是基于已经用于治疗淋巴瘤、急性淋巴细胞白血病(ALL)和/或急性髓系白血病(AML)的化疗方案。在这篇叙述性综述中,我们讨论了BPDCN的定义和流行病学特征,提供了不同的发病理论,特别注意假定的起源细胞,讨论了提供其存在迹象的主要临床表现,总结了主要的组织病理学和免疫表型特征,特别注意了最重要的标志物,最后,我们提供了一些关于BPDCN治疗方式的最有效信息。
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Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview
Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review and further studies that deeply investigate this entity not only in a histopathological but also molecular field. In August–September 2023, we searched MEDLINE, PubMed and Scopus for randomized controlled trials (RCTs), narrative and systematic reviews, meta-analyses, observational studies (either longitudinal or retrospective), and case series published in English in the last 25 years using the keywords BPDCN, PDCs, Blastic NK-cell lymphoma, agranular CD4+ NK leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm/tumor. Despite the progress made in recent years in the diagnosis and biological understanding of the disease, until 2018 there was no clear consensus regarding its treatment and the main therapeutic schemes used were based on chemotherapy regimens already used in the treatment of lymphomas, acute lymphoblastic leukemia (ALL) and/or acute myeloid leukemia (AML). In this narrative review, we address the definition and epidemiological features of BPDCN, provide the different theories on the etiopathogenesis with particular attention to the presumed cell of origin, discuss the main clinical manifestations that provide a sign of its presence, summarize the main histopathological and immunophenotypic characteristics with special attention to the most important markers, and finally, we provide some of the most effective information on the therapeutic treatment modalities of BPDCN.
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来源期刊
Hematology Reports
Hematology Reports HEMATOLOGY-
CiteScore
0.90
自引率
0.00%
发文量
47
审稿时长
10 weeks
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