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[Clinical and Pathological Features of Blastic Plasmacytoid Dendritic Cell Neoplasm].

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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Background/aim: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematologic cancer which is difficult to diagnose and has a lot of overlapping features with other diseases, particularly acute myeloid leukemia (AML). BPDCN shares several immunophenotypic markers with AML, such as CD4, CD56, CD123, and HLA-DR, stating the importance of having extending panel of specific immunohistochemical (IHC) markers.

Case Report: This report details a case of CLL who presented with worsening symptoms of recurrent infections and leukocytosis.

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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.

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