Blastic plasmacytoid dendritic cell neoplasm (Q39140): Difference between revisions
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A neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) é um tumor clinicamente agressivo derivado dos precursores de células dendríticas plasmocitoides (CDPs, também chamadas de células profissionais produtoras de interferon tipo I ou monócitos plasmocitoides), com alta frequência de envolvimento cutâneo e da medula óssea e disseminação leucêmica._x000D_ _x000D_ Atualmente, não há pistas sobre a etiologia da NCDPB , mas sua associação com síndrome mielodisplásica (SMD) em alguns casos pode sugerir uma patogênese relacionada. Estudos de perfil de expressão gênica revelaram que as células neoplásicas mostram uma assinatura de expressão gênica semelhante à de CDPs normais em repouso e mais próxima de precursores mieloides do que de linfoides. | |||
| description / en | description / en | ||
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. | |||
Revision as of 04:27, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 2A60.5 |
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| English | Blastic plasmacytoid dendritic cell neoplasm |
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. |
