Skip to main content

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

Publication ,  Journal Article
Cheng, W; Yu, T-T; Tang, A-P; He Young, K; Yu, L
Published in: Curr Med Sci
June 2021

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.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Curr Med Sci

DOI

EISSN

2523-899X

Publication Date

June 2021

Volume

41

Issue

3

Start / End Page

405 / 419

Location

China

Related Subject Headings

  • Transcription Factor 4
  • Skin Neoplasms
  • Receptors, Immunologic
  • Membrane Glycoproteins
  • Lectins, C-Type
  • Interleukin-3 Receptor alpha Subunit
  • Humans
  • Hematologic Neoplasms
  • Disease Progression
  • Dendritic Cells
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Cheng, W., Yu, T.-T., Tang, A.-P., He Young, K., & Yu, L. (2021). Blastic Plasmacytoid Dendritic Cell Neoplasm: Progress in Cell Origin, Molecular Biology, Diagnostic Criteria and Therapeutic Approaches. Curr Med Sci, 41(3), 405–419. https://doi.org/10.1007/s11596-021-2393-3

Published In

Curr Med Sci

DOI

EISSN

2523-899X

Publication Date

June 2021

Volume

41

Issue

3

Start / End Page

405 / 419

Location

China

Related Subject Headings

  • Transcription Factor 4
  • Skin Neoplasms
  • Receptors, Immunologic
  • Membrane Glycoproteins
  • Lectins, C-Type
  • Interleukin-3 Receptor alpha Subunit
  • Humans
  • Hematologic Neoplasms
  • Disease Progression
  • Dendritic Cells