Blastic plasmacytoid dendritic cell neoplasm (Q39140): Difference between revisions

@@ description / pt-br / description / pt-br @@
+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.
@@ Property / Canonical URI @@
+https://id.who.int/icd/entity/783045723
@@ Property / Canonical URI: https://id.who.int/icd/entity/783045723 / rank @@
+Normal rank
@@ Property / CURIE @@
+CID11:2A60.5
@@ Property / CURIE: CID11:2A60.5 / rank @@
+Normal rank
@@ Property / Canary Token @@
+dki-india-2A60.5
@@ Property / Canary Token: dki-india-2A60.5 / rank @@
+Normal rank
@@ Property / Verification Status @@
+Concluído
@@ Property / Verification Status: Concluído / rank @@
+Normal rank
@@ Property / Knowledge Architect @@
+https://pauloleads.com.br/cases-publicos/
+Normal rank
@@ Property / Collection date @@
+August 2026Timestamp +2026-08-13T00:00:00Z
Timezone +00:00
Calendar Gregorian
Precision 1 day
Before 0
After 0
-Timestamp
++2026-08-13T00:00:00Z
-Timezone
++00:00
-Calendar
+Gregorian
-Precision
+day
 Before
 After
@@ Property / Collection date: 13 August 2026 / rank @@
+Normal rank
@@ Property / Linked ICD 10 @@
+C86.4
@@ Property / Linked ICD 10: C86.4 / rank @@
+Normal rank

Latest 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
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.