ICD-11 definition : Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive tumour
derived from the precursors of plasmacytoid dendritic cells (PDCs, also called professional
type I interferon–producing cells or plasmacytoid monocytes), with a high frequency
of cutaneous and bone marrow involvement and leukaemic dissemination. There are currently
no clues to the etiology of BPDCN, but its association with myelodysplastic syndrome
(MDS) in some cases may suggest a related pathogenesis. Gene expression profiling
studies have revealed that the neoplastic cells show a gene expression signature similar
to that of resting normal PDCs and closer to that of myeloid than of lymphoid precursors.;
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive tumour
derived from the precursors of plasmacytoid dendritic cells (PDCs, also called professional
type I interferon–producing cells or plasmacytoid monocytes), with a high frequency
of cutaneous and bone marrow involvement and leukaemic dissemination. There are currently
no clues to the etiology of BPDCN, but its association with myelodysplastic syndrome
(MDS) in some cases may suggest a related pathogenesis. Gene expression profiling
studies have revealed that the neoplastic cells show a gene expression signature similar
to that of resting normal PDCs and closer to that of myeloid than of lymphoid precursors.
Currated CISMeF NLP mapping