Blastic plasmacytoid dendritic cell neoplasm
Synonyms: BPDCN
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Elderly
- Status
- active
A rare and highly aggressive myeloid malignancy characterized by predominant cutaneous involvement with concomitant or subsequent bone marrow, lymph node and visceral involvement, as well as central nervous system (CNS) disease. It derives from the precursors of plasmacytoid dendritic cells (pDC).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Blastic plasmacytoid dendritic cell neoplasm | BPDCN |
| fr | Tumeur à cellules plasmacytoïdes dendritiques blastiques | TCPDB |
| de | Blastische plasmazytoide dendritische Zell-Neoplasie | BPDCN · NK-Zell-Lymphom, lymphoblastoide Variante · NK-Zell-Lymphom, monomorphisches |
| es | Neoplasia blástica de células dendríticas plasmocitoides | = en |
| it | Neoplasia a cellule dendritiche plasmacitoidi blastiche | = en |
| nl | Blastaire plasmacytoïde dendritische cel-neoplasma | = en |
| pt | Neoplasia de células dendríticas plasmocitóides blásticas | = en |
| pl | Nowotwór hematodermiczny CD4+/CD56+ (blastyczny chłoniak skóry) | BPDCN · Chłoniak z blastycznych komórek NK · Jednopostaciowy chłoniak z komórek NK · Limfoblastoidalny wariant chłoniaka z komórek NK · Nowotwór z blastycznych plazmacytoidalnych komórek dendrytycznych · Nowotwór z blastycznych plazmocytoidnych komórek dendrytycznych |
| cs | CD4+/CD56+ hematodermická neoplázie | BPDCN · Blastický NK-buněčný lymfom · Blastická neoplázie z plazmocytoidních dendritických buněk · Lymfoblastoidní varianta NK-buněčného lymfomu · Monomorfní NK-buněčný lymfom |
| uk | Бластна плазмоцитоїдна дендритна клітинна пухлина | = en |
| zh | CD4+/CD56+血液皮肤肿瘤 | 母细胞性浆细胞样树状突细胞肿瘤 |
| tr | CD4 + / CD56 + hematodermik neoplazm | BPDCN · Blastik NK hücreli lenfoma · Monomorfik NK hücreli lenfoma · NK hücreli lenfomanın lenfoblastoid varyantı · Blastik plazmasitoid dendritik hücreli neoplazm |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.4 | E | specific | validated |
| ICD-11 | 2A60.5 | E | specific | validated |
| MONDO | 0019467 | E | validated | |
| MONDO | 19467 | E | validated | |
| UMLS | C1301363 | E | validated | |
| GARD | 10556 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:171918T-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 12 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2015[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.03 / 100 000 | United States | Value and class | Validated | 36946176[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86870
- This record as JSON: /data/d/86870.json