Journal
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
Volume 34, Issue 3, Pages 511-521Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.hoc.2020.01.002
Keywords
Blastic plasmacytoid dendritic cell neoplasm (BPDCN); Epigenetics; Methylation; BRD4; Azacitidine; NGS
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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 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.
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