Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma
Synonyms: Berti lymphoma · Primary cutaneous epidermotropic cytotoxic CD8+ T-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Status
- active
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing, localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (the central nervous system, lung, testes). Lymph nodes are often spared.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma | Berti lymphoma · Primary cutaneous epidermotropic cytotoxic CD8+ T-cell lymphoma |
| fr | Lymphome T cutané épidermotrope CD8+ d'évolution agressive | Lymphome agressif épidermotrope type Berti |
| de | T-Zell-Lymphom, primär kutanes, aggressives epidermotropes, CD8+ | Berti-Lymphom · Primäres kutanes epidermotrophes zytotoxisches CD8+ T-Zell-Lymphom |
| es | Linfoma primario cutáneo agresivo epidermotrópico de células T CD8+ | Linfoma de Berti · Linfoma primario cutáneo epidermotrópico de células T citotóxicas CD8+ |
| it | Linfoma cutaneo primitivo a cellule T CD8+ epidermotropo aggressivo | Linfoma cutaneo primitivo a cellule T CD8+ epidermotropo citotossico · Linfoma di Berti |
| nl | Primair cutaan agressief epidermotroop CD8+ T-cellymfoom | Bertilymfoom · Primair cutaan epidermotroop cytotoxisch CD8+ T-cellymfoom |
| pt | Linfoma primário cutâneo epidermotrópico agressivo de celúlas T CD8+ | |
| pl | Pierwotny skórny chłoniak agresywny epidermotropowy z komórek T CD8+ | Chłoniak Bertiego · Pierwotny skórny chłoniak epidermotropowy cytotoksyczny z komórek T CD8+ |
| cs | Primární kožní agresivní epidermotropní CD8+ T-buněčný lymfom | Bertiho lymfom · Primární kožní epidermotropní cytotoxický CD8+ T-buněčný lymfom |
| uk | Первинна шкірна агресивна епідермотропна CD8+ Т-клітинна лімфома | Лімфома Берті · Первинна шкірна епідермотропна цитотоксична CD8+ Т-клітинна лімфома |
| zh | 原发性皮肤侵袭性亲表皮CD8阳性细胞毒性T细胞淋巴瘤 | 原发性皮肤侵袭性亲表皮CD8阳性细胞毒性T细胞淋巴瘤 |
| tr | Primer kutanöz agresif epidermotropik CD8 + T hücreli lenfoma | Berti lenfoma · Primer kutanöz epidermotropik sitotoksik CD8 + T hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C84.5 | NTBT | attributed | validated |
| ICD-11 | 2B0Y | NTBT | index | validated |
| MONDO | 0015811 | E | validated | |
| UMLS | C4518232 | 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/178528
- This record as JSON: /data/d/178528.json