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Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review.

Publication ,  Journal Article
Shi, Y; Wang, E
Published in: Archives of pathology & laboratory medicine
April 2014

Blastic plasmacytoid dendritic cell neoplasm is a rare entity grouped with the acute myeloid leukemia-related precursor neoplasms in the 2008 World Health Organization classification. It was previously postulated to originate from natural killer cells, T cells, or monocytes but is now believed to arise from the plasmacytoid dendritic cell. The pathogenesis of blastic plasmacytoid dendritic cell neoplasm is not well understood, although the neoplasm demonstrates frequent deletion of tumor suppressor genes, including RB1, CDKN1B, CDKN2A, and TP53. Blastic plasmacytoid dendritic cell neoplasm is a clinically aggressive tumor that often initially presents as cutaneous lesions and subsequently progresses to bone marrow involvement and leukemic dissemination. It is characterized by enhanced expression of CD56, CD4, and CD123, which can be detected by flow cytometry/immunohistochemistry. The differential diagnoses include myeloid sarcoma/acute myeloid leukemia, T-cell lymphoblastic leukemia/lymphoma, NK-cell lymphoma/leukemia, and some mature T-cell lymphomas/leukemias. Patients usually respond to initial chemotherapy but often relapse. Stem cell transplant may improve survival.

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Published In

Archives of pathology & laboratory medicine

DOI

EISSN

1543-2165

ISSN

0003-9985

Publication Date

April 2014

Volume

138

Issue

4

Start / End Page

564 / 569

Related Subject Headings

  • Skin Neoplasms
  • Pathology
  • Neoplastic Stem Cells
  • Leukemia, Myelomonocytic, Acute
  • Immunophenotyping
  • Humans
  • Hematologic Neoplasms
  • Disease Progression
  • Diagnosis, Differential
  • Dendritic Cells
 

Citation

APA
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ICMJE
MLA
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Shi, Y., & Wang, E. (2014). Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review. Archives of Pathology & Laboratory Medicine, 138(4), 564–569. https://doi.org/10.5858/arpa.2013-0101-rs
Shi, Yang, and Endi Wang. “Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review.Archives of Pathology & Laboratory Medicine 138, no. 4 (April 2014): 564–69. https://doi.org/10.5858/arpa.2013-0101-rs.
Shi Y, Wang E. Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review. Archives of pathology & laboratory medicine. 2014 Apr;138(4):564–9.
Shi, Yang, and Endi Wang. “Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review.Archives of Pathology & Laboratory Medicine, vol. 138, no. 4, Apr. 2014, pp. 564–69. Epmc, doi:10.5858/arpa.2013-0101-rs.
Shi Y, Wang E. Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review. Archives of pathology & laboratory medicine. 2014 Apr;138(4):564–569.

Published In

Archives of pathology & laboratory medicine

DOI

EISSN

1543-2165

ISSN

0003-9985

Publication Date

April 2014

Volume

138

Issue

4

Start / End Page

564 / 569

Related Subject Headings

  • Skin Neoplasms
  • Pathology
  • Neoplastic Stem Cells
  • Leukemia, Myelomonocytic, Acute
  • Immunophenotyping
  • Humans
  • Hematologic Neoplasms
  • Disease Progression
  • Diagnosis, Differential
  • Dendritic Cells