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We specifically discuss the mechanisms of the pathogenesis, diagnosis, and management of blastic plasmacytoid dendritic cell neoplasm (BPDCN), a rare but aggressive haematologic malignancy characterized by frequent skin manifestations and systemic dissemination. The article enriches our understanding of BPDCN through detailed case reports showing the clinical, immunophenotypic, and histopathological features that are critical for diagnosing this disease. These cases highlight the essential role of pathologists in employing advanced immunophenotyping techniques to accurately identify the disease early in its course and guide treatment decisions. Furthermore, we explore the implications of these findings for management strategies, emphasizing the use of targeted therapies such as tagraxofusp and the potential of allogeneic haematopoietic stem cell transplantation in achieving remission. The editorial underscores the importance of interdisciplinary approaches in managing BPDCN, pointing towards a future where precision medicine could significantly improve patient outcomes.
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http://dx.doi.org/10.12998/wjcc.v12.i31.6441 | DOI Listing |
Leuk 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 PDFSci Rep
August 2025
Iranian Research Institute of Plant Protection, Agricultural Research, Education and Extension Organization (AREEO), Tehran, Iran.
Brown rot disease, caused by species of the genus Monilinia, is a significant fungal disease affecting pome and stone fruit trees. In this study, 565 samples were collected from symptomatic trees across six provinces of Iran between 2018 and 2022. A total of 430 fungal isolates were obtained and identified using both morphological and molecular techniques.
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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