Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematodermic neoplasm usually involving the skin. In this retrospective case series, 10 cases of BPDCN were identified, 90% of which had skin involvement and exhibited predominantly violaceous nodules and/or bruise-like plaques. Skin lesions showed diffuse or nodular dermal-based infiltrates of intermediate sized blasts with a Grenz zone. Tumor immunophenotyping was CD4(+), CD56(+), CD123(+) and CD303(+). The most frequently mutated genes according to targeted next-generation sequencing were TET2 (3/7) and NRAS (2/7). Multiagent chemotherapy (CT) was administered as first-line therapy, and a total of 5 patients underwent allogenic hematopoietic stem cell transplantation (allo-HSCT). Better outcomes were observed in younger patients and those treated with acute lymphoblastic leukemia (ALL)-like CT followed by allo-HSCT. This study shows the clinical range of cutaneous lesions of BPDCN. Despite the absence of a gold standard therapy, patients treated with myeloablative intensive regimens and allo-HSCT seems to have a more favorable prognosis.
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http://dx.doi.org/10.1016/j.ad.2024.11.002 | DOI Listing |
Pathology
November 2024
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. Electronic address:
Clin Pathol
December 2024
Department of Pathology and Laboratory Medicine, University of California Davis, Sacramento, CA, USA.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive neoplastic process of precursor plasmacytoid dendritic cells. The diagnostic evaluation of this heterogenous entity is challenging, requiring a comprehensive approach of incorporating clinical, morphologic, immunohistochemical, and molecular/cytogenetic evaluations. Optimal management of BPDCN remains controversial, and clinical outcomes continues to be poor.
View Article and Find Full Text PDFAsian J Surg
December 2024
Department Care Unit, Hebei Yanda Lu Daopei Hospital, Langfang, 065201, Hebei Province, China. Electronic address:
Hematology Am Soc Hematol Educ Program
December 2024
Department of Leukemia, University of Texas, MD Anderson Cancer Center, Houston, TX.
Blood Adv
December 2024
Gustave Roussy, Villejuif, France.
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