摘要
母细胞性浆细胞样树突状细胞肿瘤(BPDCN)是一种极其罕见的侵袭性肿瘤,大多数预后差.BPDCN最常见的临床表现是皮肤损伤、骨髓受累、肿瘤细胞扩散.它经常容易被误诊为其他疾病,其确诊常需要根据临床表现、影像学、组织学特征及免疫表型.其中,免疫表型的检测对BPDCN的诊断起主要作用.虽然BPDCN十分罕见,且目前尚无达成共识的一线治疗方案,但随着新药的开发以及大家对BPDCN的认识增加,有许多新药及方案可以有效治疗本病.本文总结了 BPDCN的研究背景、母细胞性浆细胞样树突状细胞的起源及BPDCN的发病机制、诊断与鉴别诊断、治疗、预后等最新研究进展.
Abstract
Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is an extremely rare invasive tumor with poor prognosis.The common clinical manifestations of BPDCN include skin injury,bone marrow involve-ment,and tumor cell spread.BPDCN is often misdiagnosed as other diseases and its diagnosis often requires a combination of clinical manifestations,imaging,histology,and immunophenotyping.Among them,immunophe-notyping is crucial for the diagnosis of BPDCN.Although BPDCN is rare and no consensus has been reached on first-line treatment option,new drugs and options for treating this disease have emerged with the development of new drugs and increased awareness of BPDCN.This article reviews the research background,the origin of blastic plasmacytoid dendritic cells,and the recent research progress in the pathogenesis,diagnosis and differential di-agnosis,treatment,and prognosis of BPDCN.
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