单中心卡斯特曼病:关于纺锤形细胞增生和侵袭性局部疾病的最新进展和新见解》(Unicentric Castleman Disease: Updates and Novel Insights into Spindle Cell Proliferations and Aggressive Forms of a Localized Disease)。

Fnu Alnoor, Alexandra Rangel, Matthew Luo, Oscar Silva, Karen M Chisholm, Dennis O'Malley, Roger Warnke, Jyoti Kumar, Robert S Ohgami
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摘要

卡斯特曼病(CD)是一种罕见的淋巴增生性疾病,可分为两种主要形式:单中心卡斯特曼病(UCD)和多中心卡斯特曼病(MCD)。单中心卡斯特曼病是局部性的,而多中心卡斯特曼病则是全身性的。虽然 UCD 在手术切除后一般预后良好,但也发现了更具侵袭性的疾病形式,包括与树突状细胞和纺锤形细胞增殖相关的病例。基因分析加深了我们对 UCD 的了解。尽管在更好地了解 UCD 的病理生理学方面取得了进展,但由于其罕见性和异质性,在诊断、管理和治疗方面仍然存在挑战。在此,我们回顾了有关 UCD 的现有知识,重点介绍了其流行病学、临床表现、诊断标准和治疗方案,同时强调了进一步研究和创新治疗策略的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Unicentric Castleman Disease: Updates and Novel Insights Into Spindle Cell Proliferations and Aggressive Forms of a Localized Disease.

Castleman Disease (CD) is a rare lymphoproliferative disorder that can be separated into two primary forms: Unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD is localized, while MCD is systemic. Though UCD generally has a favorable prognosis following surgical resection, more aggressive forms of this disease have been identified, including cases associated with dendritic and spindle cell proliferation. Genetic analysis has deepened our understanding of UCD. Despite advancements in better understanding the pathophysiology of UCD, challenges persist in the diagnosis, management, and treatment due to its rarity and heterogeneity. Here, we review current knowledge on UCD, highlighting the epidemiology, clinical presentation, diagnostic criteria, and treatment options while emphasizing the need for further research and innovation in therapeutic strategies.

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