Iranian Journal of Pathology

Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Document Type : Case Reports

Authors

Farzaneh Nayeri 1
Pardis Nematolahi 2
Behnoosh Mohamadi Jazi 3

1 Department of Pathology, Resident of Pathology, Isfahan University of Medical Sciences, Isfahan, Iran

2 Department of Pathology, Associate professor, Isfahan University of Medical Sciences, Isfahan, Iran

3 Department of Pathology, Assistant Professor, Isfahan University of Medical Sciences, Isfahan, Iran

https://doi.org/10.30699/ijp.2025.2025501.3279
Abstract
A rare condition, blastic plasmacytoid dendritic cell neoplasm, is classified as acute myeloid leukemia–related precursor neoplasms according to the World Health Organization’s 2022 classification. Previously thought to originate from natural killer cells, T cells, or monocytes, it is now believed to arise from plasmacytoid dendritic cells. The cause of this condition is not well understood, but it is often associated with the deletion of tumor suppressor genes such as RB1, CDKN1B, CDKN2A, and TP53.
The disease is aggressive and typically presents with initial cutaneous lesions that can progress to bone marrow involvement and leukemic dissemination. Flow cytometry/ immunohistochemistry can detect enhanced CD56, CD4, and CD123 expression. The differential diagnoses include myeloid sarcoma/acute myeloid leukemia, T-cell lymphoblastic leukemia/lymphoma, NK-cell lymphoma/leukemia, and certain mature T-cell lymphomas/leukemias. Although initial chemotherapy may elicit a patient response, relapse is common. Survival may be improved by stem cell transplantation.
This case report details the medical history of a 64-year-old woman who presented with a skin mass that exhibited slow growth over 6 months. The mass was firm on palpation. Extensive testing, including a bone marrow (BM) smear and biopsy, revealed numerous abnormal or blastic cells. Furthermore, flow cytometric analysis of the BM confirmed the presence of plasmacytoid dendritic cell–neoplastic precursor cells exhibiting CD4+ and CD56+ characteristics.

Keywords

Acute leukemia
Blastic plasmacytoid dendritic cell neoplasm
Chemotherapy
Stem cell transplantation

Subjects

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Iranian Journal of Pathology
Volume 20, Issue 4
Summer 2025
Pages 495-505

  • Receive Date 12 April 2024
  • Revise Date 03 February 2025
  • Accept Date 23 May 2025

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