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Updated: May 31, 2026
Assessing the Development of Murine Plasmacytoid Dendritic Cells in Peyer's Patches Using Adoptive Transfer of Hematopoietic Progenitors
Published on: March 17, 2014
Blastic plasmacytoid dendritic cell neoplasm expressing the CD13 myeloid antigen
Daichi Inoue1, Kyoko Maruyama, Kazunari Aoki
1Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, Kobe, Japan. daichi-i@hotmail.co.jp
Insights
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) can express myeloid antigen CD13, challenging previous diagnostic criteria. This rare cancer showed a positive response to chemotherapy, achieving complete remission.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy characterized by CD4+CD56+ expression.
- BPDCN is typically considered to arise from immature plasmacytoid dendritic cells (DCs), with its exact cellular origin still debated.
Observation:
- A case study of an 80-year-old male with BPDCN involving the orbital cavity and bone marrow is presented.
- The neoplastic cells in this BPDCN case strongly expressed the CD13 myeloid antigen, an unusual finding.
- In vitro, IL-3 and CD40 ligand treatment induced morphological changes and enhanced expression of DC/antigen-presenting cell markers.
Findings:
- This study reports the first instance of BPDCN expressing the CD13 myeloid antigen, though CD33 has been noted in some cases.
- The observed in vitro changes suggest a potential plasticity in BPDCN cells.
- The patient achieved complete remission after two courses of cytarabine and etoposide chemotherapy.
Implications:
- CD13 expression in BPDCN may not preclude the diagnosis, necessitating a broader understanding of BPDCN cell lineage.
- Further research into the cellular origin and potential plasticity of BPDCN is warranted.
- This case highlights the importance of considering myeloid antigen expression in the differential diagnosis of BPDCN.
Abstract:
Blastic plasmacytoid dendritic cell neoplasm (BPDCN), currently considered to originate from immature plasmacytoid dendritic cells (DC), is a rare and aggressive CD4+CD56+ neoplasm that frequently involves the skin and bone marrow. We present a case of an 80-year-old man with a CD4+CD56+ BPDCN that affected the orbital cavity and bone marrow. Although BPDCN has not been reported to express any lineage-specific markers, the neoplastic cells strongly expressed the CD13 antigen. Therefore, in addition to pathological examination, we attempted to induce in vitro morphological and surface marker changes with IL-3 and CD40 ligand. After treatment with these cytokines, the tumor cells enlarged markedly, acquired many fine dendrites, similar to mature DC, and showed enhanced expression of antigens specific to DC or antigen-presenting cells, such as CD40, CD80, CD83 and CD86. To the best of our knowledge, this is the first report of BPDCN expressing a myeloid antigen, CD13, although CD33 expression has been described in some cases. The present patient received 2 courses of combination chemotherapy consisting of cytarabine and etoposide, which resulted in complete remission. Given that the cellular origin of plasmacytoid DC is still controversial, myeloid antigen expression involving CD13 may not exclude a diagnosis of BPDCN.
