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

ABSTRACT: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy with poor outcomes. The World Health Organization (WHO) redefined BDCN as a distinct disease entity in 2016. BPDCN arises from plasmacytoid dendritic cells, manifesting primarily in the skin, bone marrow, and lymph nodes, occasionally involving the central nervous system (CNS). This presents challenges in diagnosis and treatment, with CNS involvement often overlooked in standard diagnostic workups due to BPDCN's rarity and patients often being neurologically asymptomatic at diagnosis. CNS involvement typically emerges during relapse, yet clinical trials often exclude such cases, limiting our understanding of its development and treatment. Treatment options for CNS involvement include intrathec

SUBMITTER: Mehra S 

PROVIDER: S-EPMC10854688 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature