Blastic Plasmacytoid Dendritic Cell Neoplasm

Research output: Contribution to journalReview articlepeer-review

40 Scopus citations

Abstract

Purpose of Review: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignancy derived from plasmacyoid dendritic cells whose biology, clinical features, and treatment options are increasingly better understood. Recent Findings: TCF4 is a master regulator that drives donwstream transcriptional programs in BPDCN. In turn, TCF4 activity is dependent on the bromodomain and extra-terminal domain (BET) protein BRD4 whose inhibition provides a promising therapeutic vulnerability. Notably, TCF4 expression is a highly sensitive marker for BPDCN and augments diagnostic specificity alongside CD4, CD56, CD123, and TCL1. The gene expression profile of BPDCN is characterized by aberrant NF-kappaB pathway activation, while its genomic landscape is dominated by structural chromosomal alterations involving ETV6, MYC, and NR3C1, as well as mutations in epigenetic regulators particularly TET2. Summary: Advances in elucidating the biological characteristics of BPDCN are resulting in a more refined diagnostic approach and are opening novel therapeutic avenues for patients with this disease.

Original languageEnglish (US)
Pages (from-to)477-483
Number of pages7
JournalCurrent Hematologic Malignancy Reports
Volume13
Issue number6
DOIs
StatePublished - Dec 1 2018
Externally publishedYes

Keywords

  • Acute leukemia
  • Blastic plasmacytoid dendritic cell neoplasm
  • CD123
  • Flow cytometry
  • Plasmacytoid dendritic cells
  • TCF4

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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