Molecular Features of Blastic Plasmacytoid Dendritic Cell Neoplasm: DNA Mutations and Epigenetics

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm with a dismal prognosis and no standard therapy. In the past, its cellular ontogenesis was obscure, and BPDCN had been erroneously named CD56⁺/TdT⁺ blastic NK cell tumor and CD4⁺/CD56⁺ hematodermic neoplasm. Finally, in 2008, the BPDCN was correctly recognized as a neoplasm deriving from the malignant transformation of plasmacytoid dendritic cell precursors and classified among the myeloid neoplasms. Since then, the understanding of BPDCN biology has improved rapidly: the DNA mutational status of BPDCN has been extensively investigated revealing a spectrum perfectly resembling its myeloid lineage derivation.