Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that is highly aggressive with a poor prognosis. There is no standard treatment for BPDCN. Although conventional chemotherapies are usually sensitive in the initial therapy, relapse and drug resistance are inevitable within a short duration. Targeted therapies have enlightened new prospects for the treatment of BPDCN, especially for those in a frail state and intolerable to standard chemotherapies or hematopoietic stem cell transplantation. Here, we report an 82-year-old man diagnosed with cutaneous-limited BPDCN. Considering the old age and limited involvement of the tumor, we reduced the dosage of venetoclax. His skin lesions subsided significantly after 1 cycle of azacytidine (100 mg d1-7) combined with reduced doses of venetoclax (200 mg d1-14). The reduction in the dose of venetoclax avoided severe myelosuppression while achieving satisfactory outcomes. The patient received 2 cycles of therapy with no skin lesions re-occurred for 7 months before relapsing.
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http://dx.doi.org/10.1007/s00277-024-05633-y | DOI Listing |
Acta Dermatovenerol Croat
November 2024
Prof. Miloš Nikolić, MD, PhD, University of Belgrade, School of Medicine,, Belgrade, Serbia;
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and aggressive hematologic malignancy, arising from plasmacytoid dendritic cells (pDCs). BPDCN frequently has, at least initially, exclusively cutaneous presentation. We present a 45-year-old woman with a 3-month history of rapidly evolving violaceous patches, infiltrated plaques, and bruise-like tumefactions, disseminated on her face and upper trunk.
View Article and Find Full Text PDFPediatr Blood Cancer
January 2025
Department of Pediatrics, Mie University Graduate School of Medicine, Tsu, Japan.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao
December 2024
Department of Hematology, Hebei General Hospital,Shijiazhuang 050051,China.
Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is an extremely rare invasive tumor with poor prognosis.The common clinical manifestations of BPDCN include skin injury,bone marrow involvement,and tumor cell spread.BPDCN is often misdiagnosed as other diseases and its diagnosis often requires a combination of clinical manifestations,imaging,histology,and immunophenotyping.
View Article and Find Full Text PDFCureus
December 2024
Pathology Department, Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Istanbul, TUR.
Zhongguo Shi Yan Xue Ye Xue Za Zhi
December 2024
Department of Laboratory Medicine, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
Objective: To summarize and analyze the clinical features of blastic plasmacytoid dendritic cell neoplasm (BPDCN), so as to enhance the understanding of this disease.
Methods: The clinical manifestations, immunophenotype, pathological features, treatment and prognosis of 11 cases of BPDCN were retrospectively analyzed.
Results: Among the 11 patients diagnosed with BPDCN, there were 8 males and 3 females, with a median age of 44 (6-81) years.
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