Historically, treatment options for blastic plasmacytoid dendritic cell neoplasm (BPDCN) were limited to conventional chemotherapy, adopted from regimens used to treat acute myeloid or acute lymphoblastic leukemias, or lymphomas. Nowadays, a novel therapy targeting CD123 is available to treat BPDCN. Yet, regardless of treatment choice, achieving a first complete remission (CR1) represents the main goal of therapy, because it represents the best opportunity to prolong survival in BPDCN, if offered an allogeneic hematopoietic cell transplant (allo-HCT) as consolidative therapy. Although no specific conditioning regimen is considered standard-of-care in allo-HCT eligible patients, recent data from two large registries reported a survival advantage when offering total body irradiation-based myeloablative conditioning (MAC) regimens. Unfortunately, applicability of MAC regimens is not feasible in older/unfit patients, which represents a considerable proportion of patients presenting worldwide. In such cases, reduced intensity conditioning regimens represent the next best option. Auto-HCT could be considered in older/unfit patients who did not have BM involvement at initial presentation and at time of the procedure, albeit data supporting this option is less abundant. Future research is needed to decipher the interplay between clinical, genetic, and molecular features of the disease to personalize treatment accordingly, by enhancing efficacy and avoiding unnecessary toxicities.
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Review Article|
October 7, 2024
How I treat Blastic Plasmacytoid Dendritic Cell Neoplasm
Mohamed A Kharfan-Dabaja,
Mayo Clinic, United States
* Corresponding Author; email: [email protected]
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Andrew A. Lane,
Andrew A. Lane
Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts, United States
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Naveen Pemmaraju
Naveen Pemmaraju
The University of Texas MD Anderson Cancer Center, Houston, Texas, United States
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Blood blood.2024024262.
Article history
Submitted:
July 8, 2024
Revision Received:
September 25, 2024
Accepted:
September 26, 2024
Citation
Mohamed A Kharfan-Dabaja, Andrew A. Lane, Naveen Pemmaraju; How I treat Blastic Plasmacytoid Dendritic Cell Neoplasm. Blood 2024; blood.2024024262. doi: https://doi.org/10.1182/blood.2024024262
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