Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare, primary cutaneous T-cell lymphoma characterized by solitary cutaneous nodule or only regional disease, typically occurring on the head and neck, and involving entire dermis. Sometimes, subcutis and adnexal structures are involved, as well. The infiltrate is nodular or diffuse, composed of small to medium sized pleomorphic lymphocytes and showing mild to moderate cytologic atypia. Neoplastic T-cells are mixed with B-cells, histiocytes, plasma cells and eosinophils.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma | |
| fr | Lymphome T cutané à cellules pléomorphes de taille petite à moyenne CD4+ | |
| de | T-Zell-Lymphom, primär kutanes, klein/mittelgroßzelliges pleomorphes, CD4-+ | |
| es | Linfoma cutáneo primario pleomórfico de células T CD4+ pleomórficas de pequeño y mediano tamaño | |
| it | Linfoma cutaneo pleomorfo primitivo a cellule T CD4+ di piccole-medie dimensioni | |
| nl | Primair cutaan CD4+ klein/middelgroot pleomorf T-cellymfoom | Primair cutaan CD4+ klein/middelgroot pleiomorf T-cellymfoom |
| pt | Linfoma primário cutâneo de células T CD4+ pleomórficas e de pequeno/médio tamanho | |
| pl | Pierwotny chłoniak skóry z małych/średnich pleomorficznych komórek T CD4+ | |
| cs | Primární kožní lymfom s CD4+ malými/středně velkými pleomorfními T-buňkami | |
| uk | Первинна шкірна CD4+ плеоморфних Т-клітин малих/середніх розмірів лімфома | |
| zh | 原发性皮肤CD4+多形性小/中T细胞淋巴瘤 | |
| tr | Primer kutanöz CD4 + küçük / orta boy pleomorfik 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 | 2B0Y | NTBT | index | validated |
| ICD-11 | XH7EL2 | NTBT | attributed | validated |
| MONDO | 0015810 | E | validated | |
| UMLS | C5680503 | 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:178548Indolent 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/178522
- This record as JSON: /data/d/178522.json