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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy characterized by a high frequency of cutaneous lesions at diagnosis. We report a 16-year-old female with BPDCN presenting with bruise-like macules over her face, trunk, and extremities, without evidence of other organ involvement, and review six similar cases. Pediatric BPDCN typically has a more favorable prognosis compared to adult cases. However, bruise-like lesions pose greater diagnostic challenges than other clinical presentations, such as solitary nodules, which can delay accurate diagnosis and timely treatment. Therefore, an initial diagnostic skin biopsy is valuable for early clinical and pathological evaluation, as it can support timely intervention and improve the likelihood of achieving complete biological remission.
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http://dx.doi.org/10.1111/pde.15931 | DOI Listing |
Cytometry B Clin Cytom
September 2025
Department of Hematopathology, State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, Ch
Two types of plasmacytoid dendritic cell (pDC) proliferation disease are acknowledged so far by the 5th edition of the World Health Organization Classification of Haematolymphoid Tumors: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) and mature pDC proliferation associated with myeloid neoplasms (MPDCP) in which pDC is part of the malignant clone. We aim to investigate pDC proliferation associated with non-myeloid acute leukemia (AL). A retrospective analysis of all cases admitted in our center with a diagnosis of non-myeloid AL from September 2020 to April 2023 was performed to select cases with pDCs greater than 2% of bone marrow by flow cytometry (FCM).
View Article and Find Full Text PDFLeuk Res Rep
August 2025
Division of Hematology and Hematologic Malignancies, Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.
BPDCN is an aggressive myeloid malignancy characterized by unique expression of CD123, CD4, CD56, CD303, CD304, TCL1 and TCF4. The development of tagraxofusp, a CD123-directed cytotoxin, has revolutionized BPDCN treatment, especially for patients unfit for chemotherapy. While most patients respond to frontline tagraxofusp, there are challenges associated with treatment.
View Article and Find Full Text PDFEJHaem
August 2025
Université Marie et Louis Pasteur, EFS Bourgogne Franche-Comté, INSERM, UMR RIGHT Besançon France.
Background: Venetoclax, a B-cell lymphoma 2 (BCL-2) inhibitor, is a promising treatment for blastic plasmacytoid dendritic cell neoplasm (BPDCN) and is currently under evaluation in clinical trials. However, several case reports have reported relapses after prolonged treatment, and development of resistance. To date, no study has investigated resistance to venetoclax in BPDCN.
View Article and Find Full Text PDFJ Adv Pract Oncol
July 2025
Dana-Farber Cancer Institute, Boston, Massachusetts.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive, orphan hematologic malignancy that expresses CD123 and frequently presents in skin, bone marrow, blood, and viscera. Tagraxofusp is a first-in-class CD123-targeted therapy and the only US-approved drug to treat BPDCN. Approval was based on a pivotal, multicenter, phase II study (NCT02113982), the largest prospective BPDCN trial to date, in which tagraxofusp monotherapy demonstrated durable clinical responses across treatment-naive and relapsed/refractory BPDCN, and often resulted in patients proceeding to stem cell transplant following tagraxofusp-induced remissions.
View Article and Find Full Text PDFBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological malignancy characterized by the proliferation of abnormal plasmacytoid dendritic cells. These cells infiltrate the skin and other organs during malignancy, leading to the development of violaceous skin nodules. We report a case of a middle-aged patient who presented to a secondary hospital with persistent skin lesions unresolved by primary care management and corticosteroids.
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