Annals of Hematology

, Volume 97, Issue 4, pp 563–572 | Cite as

Blastic plasmacytoid dendritic cell neoplasm: update on therapy especially novel agents

  • Shunan Wang
  • Xingtong Wang
  • Mingsuo Liu
  • Ou BaiEmail author
Review Article


Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematopoietic malignancy mainly affecting elderly patients. Most patients present with asymptomatic skin lesions as the first symptom and has a high frequency of bone marrow involvement. BPDCN is typically characterized by CD4+ and CD 56+ co-expression without common lymphoid or myeloid lineage markers. There is no consensus on the optimal therapeutic strategy for BPDCN. It is highly responsive to chemotherapy but the median event-free survival is very short. Allogeneic stem cell transplantation may improve the prognosis of BPDCN but the rate of relapse is still high. There are no specific targeted agents approved for patients with BPDCN, but advances in the understanding of the pathobiology of BPDCN and the results of early clinical studies have revealed novel targets and potentially effective agents. Novel targeted therapies may improve outcomes for patients with BPDCN in the future.


Blastic plasmacytoid dendritic cell neoplasm Hematopoietic stem cell transplantation Central nervous system prophylaxis Novel targeted agents 


Funding information

This work was supported by the development and reform commission of Jilin province, research and development of individualized information management system on malignancies with high morbidity (2014Y090).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Shunan Wang
    • 1
  • Xingtong Wang
    • 1
  • Mingsuo Liu
    • 1
  • Ou Bai
    • 1
    Email author
  1. 1.Department of HematologyThe First Hospital of Jilin UniversityChangchunPeople’s Republic of China

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