文章：

诊断母细胞性浆细胞样树突状细胞肿瘤（BPDCN）的多学科方法：来自海湾合作委员会国家的实用建议与见解

A Multidisciplinary Approach to Diagnosing Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): Practical Recommendations and Insights from Countries of the Gulf Cooperation Council

原文发布日期：11 January 2025

DOI: 10.3390/cancers17020221

类型: Article

开放获取: 是

英文摘要：

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive orphan hematopoietic malignancy characterized by cutaneous and systemic hematologic involvement. BPDCN is frequently misidentified, but early, accurate diagnosis is critical to extending patient survival using tagraxofusp, a first-in-class CD123-targeted therapy, and increasing their chances of receiving a potentially curative stem cell transplantation. Cases of BPDCN in countries of the Gulf Cooperation Council are lower than the extrapolated incidence from other geographic locations due to lack of awareness of key diagnostic features, lack of consensus on the minimal phenotype for diagnosis, and lack of local immunohistochemistry testing facilities, contributing to underdiagnosis in this region. Practical recommendations, a streamlined diagnostic panel, and suggested multidisciplinary approaches based on expert experience regarding diagnostic and clinical challenges specific to this region, and a review of the literature are presented here to facilitate diagnosis of BPDCN in this region by primary care physicians, dermatologists, and hematologists.

摘要翻译： 

母细胞性浆细胞样树突细胞肿瘤（BPDCN）是一种侵袭性罕见造血系统恶性肿瘤，以皮肤及全身血液系统受累为特征。该疾病常被误诊，但早期准确诊断对于通过使用首创CD123靶向疗法tagraxofusp延长患者生存期、提高接受潜在治愈性干细胞移植的机会至关重要。海湾合作委员会国家的BPDCN病例数低于其他地区推算的发病率，原因在于对关键诊断特征认识不足、缺乏诊断所需最低表型共识以及当地免疫组化检测设施匮乏，导致该地区疾病诊断不足。本文基于专家针对该地区特有诊断与临床挑战的经验，结合文献综述，提出实用建议、精简诊断方案及多学科协作方法，旨在帮助基层医师、皮肤科医生和血液科医生在该地区实现BPDCN的准确诊断。

原文链接：

A Multidisciplinary Approach to Diagnosing Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): Practical Recommendations and Insights from Countries of the Gulf Cooperation Council