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提高对母细胞性浆细胞样树突状细胞肿瘤的认识与管理:近期病例研究的见解

Advancing the understanding and management of blastic plasmacytoid dendritic cell neoplasm: Insights from recent case studies.

作者信息

Luo Yan, Wang Li-Juan, Wang Cheng-Long

机构信息

Department of Stomatology, The People's Hospital of Dadukou District, Chongqing 400084, China.

Department of Pathology, The Chongqing Hospital of Traditional Chinese Medicine, Chongqing 400021, China.

出版信息

World J Clin Cases. 2024 Nov 6;12(31):6441-6446. doi: 10.12998/wjcc.v12.i31.6441.

Abstract

We specifically discuss the mechanisms of the pathogenesis, diagnosis, and management of blastic plasmacytoid dendritic cell neoplasm (BPDCN), a rare but aggressive haematologic malignancy characterized by frequent skin manifestations and systemic dissemination. The article enriches our understanding of BPDCN through detailed case reports showing the clinical, immunophenotypic, and histopathological features that are critical for diagnosing this disease. These cases highlight the essential role of pathologists in employing advanced immunophenotyping techniques to accurately identify the disease early in its course and guide treatment decisions. Furthermore, we explore the implications of these findings for management strategies, emphasizing the use of targeted therapies such as tagraxofusp and the potential of allogeneic haematopoietic stem cell transplantation in achieving remission. The editorial underscores the importance of interdisciplinary approaches in managing BPDCN, pointing towards a future where precision medicine could significantly improve patient outcomes.

摘要

我们专门讨论了母细胞性浆细胞样树突状细胞肿瘤(BPDCN)的发病机制、诊断及治疗方法,这是一种罕见但侵袭性强的血液系统恶性肿瘤,其特征是频繁出现皮肤表现和全身播散。本文通过详细的病例报告丰富了我们对BPDCN的认识,这些报告展示了对诊断该疾病至关重要的临床、免疫表型和组织病理学特征。这些病例突出了病理学家在运用先进免疫表型分析技术以在疾病早期准确识别并指导治疗决策方面的重要作用。此外,我们探讨了这些发现对治疗策略的影响,强调了使用如他格拉索普等靶向疗法以及异基因造血干细胞移植实现缓解的潜力。这篇社论强调了跨学科方法在管理BPDCN中的重要性,指出精准医学的未来有望显著改善患者预后。

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