Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy that frequently has cutaneous manifestations. The diagnosis can be a challenge because of its heterogenous clinical presentation, ranging from a brown or violaceous solitary nodule or patch to mixed, disseminated lesions. Furthermore, BPDCN tumor cells express immunohistochemical markers in common with acute myeloid leukemia, which can lead to misdiagnosis. Timely diagnosis requires awareness of its cutaneous manifestations and unique histopathology and immunophenotype. We present a case series of patients diagnosed with BPDCN and review the cutaneous and histopathologic characteristics of this uncommon entity.
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http://dx.doi.org/10.1097/DAD.0000000000001968 | DOI Listing |
Cancers (Basel)
January 2025
ARUP Laboratories, Department of Pathology, University of Utah, Salt Lake City, UT 84103, USA.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive orphan hematopoietic malignancy characterized by cutaneous and systemic hematologic involvement. BPDCN is frequently misidentified, but early, accurate diagnosis is critical to extending patient survival using tagraxofusp, a first-in-class CD123-targeted therapy, and increasing their chances of receiving a potentially curative stem cell transplantation. Cases of BPDCN in countries of the Gulf Cooperation Council are lower than the extrapolated incidence from other geographic locations due to lack of awareness of key diagnostic features, lack of consensus on the minimal phenotype for diagnosis, and lack of local immunohistochemistry testing facilities, contributing to underdiagnosis in this region.
View Article and Find Full Text PDFActa 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
March 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.
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