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

Abstract

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.

• Authors: Wei Cheng, Tian-Tian Yu, Ai-Ping Tang, Ken He Young, Li Yu
• Journal: Current medical science (Curr Med Sci) Vol. 41 Issue 3 Pg. 405-419 (Jun 2021) ISSN: 2523-899X [Electronic] China
• PMID: 34218354 (Publication Type: Journal Article, Review)
• Copyright: © 2021. The Author(s).

Chemical References

• Antigens, Surface
• CD4 Antigens
• CD56 Antigen
• CLEC4C protein, human
• IL3RA protein, human
• Interleukin-3 Receptor alpha Subunit
• Lectins, C-Type
• Membrane Glycoproteins
• Receptors, Immunologic
• TCF4 protein, human
• Transcription Factor 4
• blood dendritic cell antigen 4, human

Topics

• Acute Disease (epidemiology)
• Antigens, Surface (genetics)
• CD4 Antigens (genetics)
• CD56 Antigen (genetics)
• Cell Differentiation (genetics)
• Dendritic Cells (metabolism, pathology)
• Disease Progression
• Hematologic Neoplasms (genetics, pathology)
• Humans
• Interleukin-3 Receptor alpha Subunit (genetics)
• Lectins, C-Type (genetics)
• Membrane Glycoproteins (genetics)
• Receptors, Immunologic (genetics)
• Skin Neoplasms (diagnosis, genetics, pathology, therapy)
• Transcription Factor 4 (genetics)