Clinical Features and Prognosis of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Chinese Cohort Study.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic cancer, characterized by frequent cutaneous involvement and dismal prognosis. While skin manifestations often precede systemic spread, diagnostic delays persist due to clinical mimicry of benign dermatologic conditions. Current knowledge gaps exist regarding its clinical behaviour in Chinese populations. This single-center retrospective case series was conducted to analyze the clinical characteristics and survival outcomes of BPDCN patients in China. A total of 18 patients (mean age 45.6 years; 77.8% male) were included. Cutaneous lesions were observed in 94.4% of cases, with most showing disseminated skin involvement at diagnosis. Leukemic infiltration was the most common extracutaneous manifestation, while no central nervous system involvement was detected among patients who underwent lumbar puncture. Systemic chemotherapy achieved an objective response in 85.7% of patients, but 75% experienced relapse or disease progression. The median overall survival was 13 months, with 1- and 2-year survival rates of 53.3% and 26.7%, respectively. Survival outcomes were not significantly associated with clinical features, genetic profiles, or treatment regimens. This study demonstrates the clinical diversity of BPDCN and highlights its uniformly poor prognosis regardless of therapeutic approach. The cohort had a younger age of onset than previously reported. While some patients initially presented with solitary skin lesions, most showed disseminated skin and multi-organ involvement by diagnosis. No CNS involvement was observed, and no factors associated with favorable prognosis were identified. Enhanced diagnostic strategies and the development of novel targeted therapies are urgently needed for this understudied population.