TY - JOUR
T1 - Molecular Features of Blastic Plasmacytoid Dendritic Cell Neoplasm
T2 - DNA Mutations and Epigenetics
AU - Sapienza, Maria Rosaria
AU - Pileri, Stefano
N1 - Publisher Copyright:
© 2020 Elsevier Inc.
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/6
Y1 - 2020/6
N2 - 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.
AB - 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.
KW - Azacitidine
KW - Blastic plasmacytoid dendritic cell neoplasm (BPDCN)
KW - BRD4
KW - Epigenetics
KW - Methylation
KW - NGS
UR - http://www.scopus.com/inward/record.url?scp=85081286940&partnerID=8YFLogxK
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U2 - 10.1016/j.hoc.2020.01.002
DO - 10.1016/j.hoc.2020.01.002
M3 - Review article
C2 - 32336416
AN - SCOPUS:85081286940
VL - 34
SP - 511
EP - 521
JO - Hematology/Oncology Clinics of North America
JF - Hematology/Oncology Clinics of North America
SN - 0889-8588
IS - 3
ER -