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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… Click to show full abstract
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.
Journal Title: Hematology/oncology clinics of North America
Year Published: 2020
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