Blastic Plasmacytoid Dendritic Cell Neoplasm:Progress in Cell Origin,Molecular Biology,Diagnostic Criteria and Therapeutic Approaches  

作　　者：Wei CHENG Tian-tian YU Ai-ping TANG Ken HE YOUNG Li YUI 

机构地区：[1]Department of Hematology,the Second Affiliate Hospital of Nanchang University,Nanchang 330006,China [2]Division of Hematopathology and Department of Pathology,Duke University Medical Center,Durham 27710,USA

出　　处：《Current Medical Science》2021年第3期405-419,共15页当代医学科学（英文）

基　　金：the National NaOiral Science Foundation of China(No.81460030,81770221).

摘　　要：Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is a rare hematological malignancy characterized by recurrent skin nodules,an aggressive clinical course with rapid involvement of hematological organs,and a poor prognosis with poor overall survival.BPDCN is derived from plasmacytoid dendritic cells(pDCs)and its pathogenesis is unclear.The tumor cells show aberrant expression of CD4,CD56,interleukin-3 receptor alpha chain(CD 123),blood dendritic cell antigen 2(BDCA 2/CD303),blood dendritic cell antigen 4(BDCA4)and transcription factor(E protein)E2-2(TCF4).The best treatment drugs are based on experience by adopting those used for either leukemia or lymphoma.Relapse with drug resistance generally occurs quickly.Stem cell transplantation after the first complete remission is recommended and tagraxofusp is the first targeted therapy.In this review,we summarize the differentiation of BPDCN from its cell origin,its connection with normal pDCs,clinical characteristics,genetic mutations and advances in treatment of BPDCN.This review provides insights into the mechanisms of and new therapeutic approaches for BPDCN.

关 键 词：blastic plasmacytoid dendritic cell neoplasm plasmacytoid dendritic cell genetic mutations IMMUNOPHENOTYPE THERAPEUTICS 

分 类 号：R73[医药卫生—肿瘤]