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Blastic Plasmacytoid Dendritic Cell Neoplasm-Current Insights.

Sangeetha Venugopal1, Selena Zhou2, Siraj M El Jamal3

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Clinical Lymphoma, Myeloma & Leukemia
|July 9, 2019
PubMed
Summary

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare leukemia. This review covers BPDCN etiology, diagnosis, and treatments, including the novel therapy tagraxofusp.

Keywords:
Acute leukemiaCD123Myeloid neoplasmsSL-401Tagraxofusp

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy.
  • BPDCN exhibits heterogeneous presentation and clinical course, recognized as a distinct entity by the WHO in 2016.
  • Advances in understanding BPDCN pathobiology have improved diagnostics and targeted therapies.

Purpose of the Study:

  • To review the etiology of BPDCN.
  • To summarize current diagnostic criteria for BPDCN.
  • To discuss historical and novel therapeutic strategies for BPDCN.

Main Methods:

  • Literature review of etiology, diagnosis, and treatment of BPDCN.
  • Analysis of recent advancements in BPDCN research.
  • Focus on the impact of tagraxofusp approval.

Main Results:

  • Etiology of BPDCN is complex, involving precursor cells.
  • Diagnostic criteria have been refined by WHO classification.
  • Tagraxofusp (SL-401) represents a significant novel therapeutic option.

Conclusions:

  • BPDCN requires a comprehensive understanding of its multifaceted nature.
  • Accurate diagnosis and tailored treatment are crucial for managing BPDCN.
  • Novel therapies like tagraxofusp offer new hope for BPDCN patients.