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

被引：15

作者：

Cheng, Wei ^{[1
]}

Yu, Tian-tian ^{[1
]}

Tang, Ai-ping ^{[1
]}

He Young, Ken ^{[2
,3
]}

Yu, Li ^{[1
]}

机构：

[1] Nanchang Univ, Affiliate Hosp 2, Dept Hematol, Nanchang 330006, Jiangxi, Peoples R China

[2] Duke Univ, Med Ctr, Div Hematol, Durham, NC 27710 USA

[3] Duke Univ, Med Ctr, Dept Pathol, Durham, NC 27710 USA

来源：

CURRENT MEDICAL SCIENCE | 2021年 / 41卷 / 03期

基金：

中国国家自然科学基金;

关键词：

blastic plasmacytoid dendritic cell neoplasm; plasmacytoid dendritic cell; genetic mutations; immunophenotype; therapeutics; WORLD-HEALTH-ORGANIZATION; TRANSCRIPTION FACTOR E2-2; LEUKEMIC PRESENTATION; SEQUENCING REVEALS; MYELOID NEOPLASMS; IN-VITRO; T-CELLS; EXPRESSION; MUTATIONS; RECURRENT;

D O I：

10.1007/s11596-021-2393-3

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

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 (CD123), 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.

引用

页码：405 / 419

页数：15

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