Age, race, and sex have significant implications for survival rates of patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN), according to data presented at the 2023 ASH Annual Meeting & Exposition (Abstract 5185).
Findings from the largest U.S. population–based BPDCN study revealed a population-level incidence of 0.05 cases per 100,000 and showed that older age and Black race are negative prognostic factors for overall survival in this rare hematologic malignancy. BPDCN is an aggressive but poorly understood disease that arises from dendritic cell precursors. As the authors of the study underscored, it often presents significant diagnostic and therapeutic challenges due to its rarity and changing nomenclature and defining criteria—which makes population-based studies a crucial source of information.
“Our research attempts to bring more clarity and understanding to this challenging disease,” said lead study author Ujjwal Karki, MBBS, Corewell Health William Beaumont University Hospital, in Royal Oak, Michigan, who noted that the racial disparities in overall survival warrant further investigation. “More prospective studies are needed to determine disease characteristics and optimal treatment approaches for this rare life-threatening hematologic malignancy.”
Shedding Light on a Rare, Lethal Malignancy
For this comprehensive, large-scale study, Dr. Karki and colleagues used the Surveillance Epidemiology and End Results (SEER) database to identify patients diagnosed with BPDCN between 2000 and 2019. The researchers aimed to determine the incidence, clinical characteristics, overall survival, and prognostic factors of BPDCN.
The most common primary site of disease involvement was the lymph nodes (56.6%), followed by bone marrow (19.1%) and skin (13%). In terms of treatment, a substantial majority of patients (83.6%) received chemotherapy for BPDCN, whereas a smaller group (18%) received radiation therapy.
As Dr. Karki reported, the overall incidence of BPDCN was found to be 0.05 cases per 100,000 population. Of note, the incidence was shown to be significantly lower in females than males, with an incident rate ratio of 0.62 (P = .01), and in Black patients compared to White patients, with an incident rate ratio of 0.70 (P = .01).
Survival rates at 1 year, 2 years, and 5 years were found to be 70%, 56%, and 41%, respectively. On univariate analysis, age greater than 60 years and male sex were associated with significantly worse overall survival. Although overall survival did not differ by race according to univariate analysis, after adjusting for age, sex, race, marital status, radiation therapy, chemotherapy, and disease primary site, age greater than 60 and Black race were found to be associated with worse overall survival, said Dr. Karki.
“These results underscore the importance of considering demographic factors while diagnosing and treating BPDCN and indicate the need for additional research,” said Dr. Karki. “Further exploration of the genetic and pathologic factors involved in BPDCN may provide a more tailored approach in the future.”
Disclosure: The study authors reported no potential conflicts of interest.
