Primary Blastic Plasmacytoid Dendritic Cell Neoplasm: a US Population-Based Study

BackgroundBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and poorly understood hematopoietic malignancy. This study aimed to investigate the clinical characteristics and prognostic factors in patients with primary BPDCN. MethodsPatients diagnosed with primary BPDCN from 2000 to 2019 were extracted from the Surveillance, Epidemiology and End Results (SEER) database. Independent prognostic factors were evaluated based on the univariate and multivariate Cox regression analysis. The nomogram was then constructed to predict the overall survival of primary BPDCN patients at 3, 5 and 10 years after diagnosis. ResultsA total of 668 primary BPDCN patients were included in this study. The average age was 35.7 {+/-} 25.4 years, with 68.7% being male. The mostly affected sites were lymph nodes (59.9%). Most patients (69.9%) received chemotherapy or radiation therapy. For all the patients, the 1-year, 3-year, 5-year, and 10-year overall survival (OS) were 78.0%, 62.6%, 59.0%, and 56.3%, respectively, and the corresponding disease-specific survival (DSS) were 80.6%, 66.8%, 63.5, and 61.8%, respectively. Multivariate cox analysis indicated that age and marital status of other (divorced, widowed and separated) at diagnosis were independent prognostic factors for DSS, but only age was for OS. The 5-year OS rate significantly declined with increasing age: age <15, 89.3%; age 15-39, 57.9%; age 40-64, 51.2%; age [&ge;]65, 26.1%. Nomograms were further constructed to predict the possibility of OS and DSS with good performances. ConclusionsPrimary BPDCN is a rare disease, age and marital status were associated with survival of primary BPDCN patients, and age was an independent prognostic factor for OS.