Incidence, prognostic factors, and survival outcomes in patients with blastic plasmacytoid dendritic cell neoplasm: A retrospective study in the Surveillance, Epidemiology, and End Results database

Abstract

There are few large‐scale epidemiological and prognostic studies on blastic plasmacytoid dendritic cell neoplasm (BPDCN) due to its rarity. We used the Surveillance, Epidemiology, and End Results database to investigate the incidence, clinical characteristics, prognostic factors, and survival trends of BPDCN. The age‐adjusted incidence of BPDCN had a bimodal pattern with peaks in those under 20 and 60 years and older. Of 697 patients, the median age at diagnosis was 31 years. The most common primary sites were lymph nodes (59.4%), followed by bone marrow (17.1%) and skin (11.6%). Extranodal involvement (59.7%) was more common in patients aged 60 years and older, while lymph node involvement was predominant in other age groups. The 1‐year, 3‐year, and 5‐year overall survival (OS) rates were 90.7%, 83.7%, and 82.3% in patients aged under 20, but dropped to 53.1%, 27.7%, and 20.0% in patients aged 60 and older. Multivariate Cox regression analysis revealed that age, sex, and first malignancy were independent prognostic factors for OS. Based on this regression model, a nomogram was built with high discrimination and calibration. The incidence, clinical characteristics, and prognosis of BPDCN patients vary by age group. Moreover, using the nomogram to predict OS can help guide individualized evaluations and clinical decisions.

Multiple Genomic Alterations, Including a Novel AFF4::IRF1 Fusion Gene, in a Treatment-Refractory Blastic Plasmacytoid Dendritic-Cell Neoplasm: A Case Report and Literature Review

“…In a retrospective study, age > 60 years at diagnosis, abnormal karyotype, and terminal deoxynucleotidyl transferase negativity were suggested to be associated with worse treatment responses [ 47 ]. In another study, multivariate Cox regression analysis revealed that advanced age, male gender, and BPDCN occurring as the first malignancy were independent prognostic factors for overall survival [ 48 ]. Yin et al suggested that older age, multiple mutations, and mutations in the DNA methylation pathway were poor prognostic factors for BPDCN [ 49 ].…”

Blastic Plasmacytoid Dendritic Cell Neoplasm: A Comprehensive Review of the Disease, Central Nervous System Presentations, and Treatment Strategies

“…pDCs are largely responsible for viral defense, as upon maturation, these cells are responsible for secreting cytokines such as type I interferon (IFN), interleukin-6, interleukin-8, and tumor necrosis alpha. This allows for the activation of T-cells, macrophages, and NK cells [1]. Initially, BPDCN was thought to be an NK cell malignancy due to presence of CD4+ and CD56+ cells when immunotyping of cells was conducted.…”

“…Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy primarily afflicting middle-aged to older men and has poor survival outcomes [1,2]. Although the median age of diagnosis is around 65 years of age, BPDCN has also been seen in younger populations, including children in a bimodal distribution pattern.…”

Real-world evidence on tagraxofusp for blastic plasmacytoid dendritic cell neoplasm – collected cases from a single center and case reports

“…With a reported incidence of 0.04 cases per 100.000 in the US, it represents less than 1% of all hematologic malignancies. The median age of onset of the adult variant is around 70 years and men are more frequently affected ( 2 ). The typical presentation consists of characteristic skin lesions as well as affection as leukemia or of lymph nodes, liver, spleen and, less frequently, other extramedullary sites such as the central nervous system (CNS).…”