PDF(Pubmed)
Abstract：
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematological malignancy derived from the precursors of plasmacytoid dendritic cells. Although disease awareness has increased over time, BPDCN represents a rare disease with an aggressive clinical course and a dismal prognosis. Due to the overlap in clinical and histological features with a large spectrum of inflammatory and neoplastic diseases, BPDCN is difficult to diagnose. Furthermore, given the rarity of the disease, treatment options for BPDCN are limited, sometimes changing by practitioner and hospitals. Treatment options range from conventional chemotherapy to the recently approved biologic agent tagraxofusp and stem cell transplantation. Therefore, a multidisciplinary approach with coordination among dermatologists, pathologists, and hematologists is ultimately imperative to reach the correct diagnosis and management of BPDCN.
摘要：
母细胞性浆细胞样树突状细胞肿瘤(BPDCN)是来源于浆细胞样树突状细胞前体的侵袭性血液恶性肿瘤。尽管随着时间的推移，人们对疾病的认识有所增加，BPDCN代表一种罕见的疾病，具有侵袭性的临床过程和令人沮丧的预后。由于临床和组织学特征与大量炎症和肿瘤疾病的重叠，BPDCN难以诊断。此外，鉴于这种疾病的罕见性，BPDCN的治疗选择有限，有时由医生和医院改变。治疗选择范围从常规化疗到最近批准的生物制剂tagraxofusp和干细胞移植。因此,多学科的方法与皮肤科医生之间的协调，病理学家,血液学家最终必须达到BPDCN的正确诊断和管理。
