Blastic Plasmacytoid Dendritic Cell Neoplasm-Current Insights

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare clonal hematologic malignancy of plasmacytoid dendritic cell precursors. The presentation and clinical course of BPDCN is widely heterogeneous and was most recently categorized as a distinct clinical entity by the World Health Organization in 2016. The expanded understanding of the pathobiology of BPDCN has improved diagnostic accuracy and informed novel targeted therapeutic options. The United States Food and Drug Administration-approval of tagraxofusp (SL-401) in December 2018 has focused attention on this leukemia frequently associated with skin involvement. Herein, we aim to: (1) review etiology; (2) summarize diagnostic criteria; and (3) discuss historic treatments and novel therapies for BPDCN.