Blastic Plasmacytoid Dendritic Cell Neoplasm: Progress in Cell Origin, Molecular Biology, Diagnostic Criteria and Therapeutic Approaches

SummaryBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy characterized by recurrent skin nodules, an aggressive clinical course with rapid involvement of hematological organs, and a poor prognosis with poor overall survival. BPDCN is derived from plasmacytoid dendritic cells (pDCs) and its pathogenesis is unclear. The tumor cells show aberrant expression of CD4, CD56, interleukin-3 receptor alpha chain (CD123), blood dendritic cell antigen 2 (BDCA 2/CD303), blood dendritic cell antigen 4 (BDCA4) and transcription factor (E protein) E2-2 (TCF4). The best treatment drugs are based on experience by adopting those used for either leukemia or lymphoma. Relapse with drug resistance generally occurs quickly. Stem cell transplantation after the first complete remission is recommended and tagraxofusp is the first targeted therapy. In this review, we summarize the differentiation of BPDCN from its cell origin, its connection with normal pDCs, clinical characteristics, genetic mutations and advances in treatment of BPDCN. This review provides insights into the mechanisms of and new therapeutic approaches for BPDCN.

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A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Extensively Studied by Flow Cytometry and Immunohistochemistry

Case Reports in Hematology ◽

10.1155/2017/4984951 ◽

2017 ◽

Vol 2017 ◽

pp. 1-6

Author(s):

Martina Pennisi ◽

Clara Cesana ◽

Micol Giulia Cittone ◽

Laura Bandiera ◽

Barbara Scarpati ◽

...

Keyword(s):

Flow Cytometry ◽

Dendritic Cell ◽

Hematologic Malignancy ◽

Lymphoblastic Leukemia ◽

Plasmacytoid Dendritic Cell ◽

Bone Marrow Involvement ◽

Hematopoietic Stem ◽

Cell Neoplasm ◽

Specific Antigens ◽

Aggressive Clinical Course

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4+CD56+,CD123high, TCL-1+, and blood dendritic cell antigen-2/CD303+blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete remission after acute lymphoblastic leukemia like chemotherapy and allogeneic hematopoietic stem cell transplantation.

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Blastic plasmacytoid dendritic-cell neoplasia: a challenging case report

Journal of Cancer Research and Clinical Oncology ◽

10.1007/s00432-021-03777-2 ◽

2021 ◽

Author(s):

Ruth-Miriam Koerber ◽

Stefanie A. E. Held ◽

Maria Vonnahme ◽

Georg Feldmann ◽

Joerg Wenzel ◽

...

Keyword(s):

Dendritic Cell ◽

Myeloid Leukemia ◽

Plasmacytoid Dendritic Cell ◽

Correct Diagnosis ◽

Normal Karyotype ◽

Standards Of Care ◽

Poor Overall Survival ◽

Bone Marrow Biopsies ◽

Cell Neoplasm ◽

Myelomonocytic Leukemia

AbstractBlastic plasmacytoid dendritic-cell neoplasm (BPDCN) is an extremely rare disease that originates from dendritic cells and is associated with a poor overall survival (OS). Diagnostic and therapeutic standards are less well-established in comparison to other leukemic conditions and standards of care are lacking. Morphologic and molecular similarities to acute myeloid leukemia (AML), myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia (CMML) are hard to distinguish. We here report a BPDCN patient with a long, challenging diagnostic period. While bone marrow biopsies initially failed to prove the correct diagnosis, a cutaneous biopsy finally identified a CD45+/CD56+/CD4+/CD123+/CD33+/MPO− population suggestive of BPDCN which was confirmed by flow cytometry. Molecular analysis revealed an ASXL-1, TET2 and SRSF2-mutation, cytogenetic analysis showed a normal karyotype. Treatment with the recently approved CD123-cytotoxin Tagraxofusp showed initially a very good response. This case reflects diagnostic and therapeutic difficulties in BPDCN as very rare, easily misdiagnosed neoplasia and the need for precise diagnostic care.

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Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report of a Rare Leukemic Variant

10.31487/j.jcmcr.2020.01.03 ◽

2020 ◽

pp. 1-3

Author(s):

Ammar Al-Obaidi ◽

Chloe McBride ◽

Christopher Dakhil ◽

Khalil Choucair ◽

...

Keyword(s):

Dendritic Cell ◽

Hematological Malignancies ◽

Hematological Malignancy ◽

Early Recognition ◽

Systemic Disease ◽

Plasmacytoid Dendritic Cell ◽

Rapid Progression ◽

Cell Neoplasm ◽

Aggressive Clinical Course ◽

Older Males

Blastic plasmacytoid dendritic cell neoplasm, originally described in 1995, is a very rare hematological malignancy with an aggressive clinical course characterized by a rapid progression to systemic disease via hematogenous dissemination. It represents around 0.5% of all hematologic cancers and typically affects older males. Early recognition remains a challenge as it largely overlaps with other hematological malignancies.

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