Does H3K27me3 expression play a role in patients with Blastic plasmacytoid dendritic cell neoplasm? A clinicopathologic analysis of 14 patients

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive lymphohematopoietic malignancy associated with poor prognosis. We aimed to improve the understanding of BPDCN, explore its prognostic significance, and identify potential therapeutic targets. Data from 14 BPDCN patients were retrospectively collected and analyzed, focusing on their clinicopathological characteristics, diagnostic features, immunophenotype, treatment regimens, and prognostic factors. Additionally, immunohistochemistry was used to detect the expression of multiple oncogenes in BPDCN. The cohort comprised 14 patients (10 males, 4 females) with a median age of 63.5 years at the time of diagnosis. Of these specimens, H3K27me3, ASXL1, BAP1, RAC1, TCF4 and AURKA were highly expressed in BPDCN, with expression rates of 71.4 % (10/14), 92.9 % (13/14), 85.7 % (12/14), 100 % (13/13), 12/14 (85.7 %) and 46.2 % (6/13), respectively. The survival of patients in this cohort ranged from 1 to 84 months, with a median overall survival (OS) of 18.5 months. The survival rates for 1, 2, 3, 4 and 5 years were 71.43 %, 53.57 %, 44.64 %, 44.64 %, and 44.64 %, respectively. In the overall BPDCN cohort, patients with positive expression of H3K27me3 exhibited significantly better overall survival compared to those with negative expression H3K27me3 (P = 0.0056). Our analysis showed that the absence of H3K27me3 expression may indicate a poor prognosis in patients with BPDCN, and H3K27me3 may be a potential prognostic indicator for BPDCN.