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

IF 1 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL World Journal of Clinical Cases Pub Date : 2024-11-06 DOI:10.12998/wjcc.v12.i31.6441
Yan Luo, Li-Juan Wang, Cheng-Long Wang
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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.

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推进对疱性浆细胞树突状细胞肿瘤的认识和管理:近期病例研究的启示。
我们特别讨论了大疱性浆细胞树突状细胞瘤(BPDCN)的发病机制、诊断和治疗方法,这是一种罕见的侵袭性血液恶性肿瘤,以频繁的皮肤表现和全身播散为特征。文章通过详细的病例报告,展示了对诊断这种疾病至关重要的临床、免疫表型和组织病理学特征,丰富了我们对 BPDCN 的认识。这些病例凸显了病理学家在运用先进的免疫分型技术在疾病早期准确识别疾病并指导治疗决策方面的重要作用。此外,我们还探讨了这些发现对治疗策略的影响,强调了靶向疗法(如 tagraxofusp)的使用以及异基因造血干细胞移植在实现缓解方面的潜力。这篇社论强调了跨学科方法在管理BPDCN中的重要性,并指出未来精准医学将显著改善患者的预后。
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World Journal of Clinical Cases
World Journal of Clinical Cases Medicine-General Medicine
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期刊介绍: The World Journal of Clinical Cases (WJCC) is a high-quality, peer reviewed, open-access journal. The primary task of WJCC is to rapidly publish high-quality original articles, reviews, editorials, and case reports in the field of clinical cases. In order to promote productive academic communication, the peer review process for the WJCC is transparent; to this end, all published manuscripts are accompanied by the anonymized reviewers’ comments as well as the authors’ responses. The primary aims of the WJCC are to improve diagnostic, therapeutic and preventive modalities and the skills of clinicians and to guide clinical practice in clinical cases.
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