Primary cutaneous peripheral T-cell lymphoma not otherwise specified
Synonyms: Primary cutaneous peripheral T-cell lymphoma NOS · Primary cutaneous unspecified peripheral T-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
An extremely rare, primary cutaneous T-cell lymphoma disorder characterized by solitary, or multifocal and diffuse, cutaneous lesions, ranging from tumor-like patches, plaques, papules, nodules, and/or erythroderma, located on any area of the body, which rapidly progress and may become ulcerated and/or infected. Systemic involvement may be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous peripheral T-cell lymphoma not otherwise specified | Primary cutaneous peripheral T-cell lymphoma NOS · Primary cutaneous unspecified peripheral T-cell lymphoma |
| fr | Lymphome cutané primitif à cellules T périphérique sans autre indication | Lymphome cutané primitif à cellules T périphérique SAI · Lymphome cutané primitif à cellules T périphérique non spécifié |
| de | Primär kutanes peripheres T-Zell-Lymphom, andernorts nicht klassifiziert | PTCL-NOS · Primär kutanes PTCL ohne weitere Spezifizierung · T-Zell-Lymphom, peripheres, primär kutanes, unspezifisches |
| es | Linfoma cutáneo primario de células T periféricas sin otra especificación | Linfoma cutáneo primario de células T periféricas inespecífico |
| it | Linfoma cutaneo periferico aspecifico primitivo a cellule T | |
| nl | Primair cutaan perifeer T-cellymfoom niet anders gespecificeerd | Primair cutaan niet-gespecificeerd perifeer T-cellymfoom · Primair cutaan perifeer T-cellymfoom NOS |
| pt | Linfoma de células T periféricas, inespecífico | |
| pl | Pierwotny chłoniak skórny z obwodowych komórek T, bliżej nieokreślony | Pierwotny skórny chłoniak z obwodowych komórek T NOS |
| cs | Primární kožní nespecifikovaný periferní T-buněčný lymfom | = en |
| uk | Первинна шкірна периферична Т-клітинна лімфома, не спеціфікована | Первинна шкірна периферична Т-клітинна лімфома NOS · Первинна шкірна неспецифічна периферична Т-клітинна лімфома |
| zh | 原发性皮肤外周T细胞淋巴瘤，未分类 | 原发性皮肤未分类外周T淋巴细胞白血病 |
| tr | Primer kutanöz periferik T hücreli lenfoma, başka türlü tanımlanmamış | Primer kutanöz periferik T hücreli lenfoma NOS · Primer kutanöz tanımlanmamış periferik T hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C84.4 | NTBT | attributed | validated |
| ICD-11 | 2A90.C | E | specific | validated |
| MONDO | 0019476 | E | validated | |
| MeSH | D016411 | E | validated | |
| UMLS | C0079774 | E | validated | |
| MedDRA | 10034623 | 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:178551Aggressive primary cutaneous T-cell lymphoma
rare skin diseases; 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
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86885
- This record as JSON: /data/d/86885.json