Primary cutaneous diffuse large B-cell lymphoma, leg type
Synonyms: PCDLBCL,LT
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare, aggressive, primary cutaneous B-cell lymphoma characterized by rapidly progressive, red to bluish, often ulcerating, nodular tumors predominantly involving the lower legs. Histology shows sheets of centroblasts and immunoblasts that spare the epidermis, but infiltrate the dermis and subcutaneous tissues, and often disseminate extracutaneously. The neoplastic cells typically express CD20, CD79a, Bcl-2, MUM-1, and FOXP1, but are negative for CD10.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous diffuse large B-cell lymphoma, leg type | PCDLBCL,LT |
| fr | Lymphome cutané à grandes cellules de type jambe | |
| de | B-Zell-Lymphom, diffuses großzelliges, primär kutanes, vom leg Typ | |
| es | Linfoma difuso cutáneo primario de células B grandes tipo pierna | |
| it | Linfoma cutaneo primitivo diffuso a grandi cellule B, tipo gamba | |
| nl | Primair cutaan diffuus grootcellig B-cellymfoom, been-type | = en |
| pt | Linfoma primário cutâneo difuso de células B de grande tamanho, tipo perna | |
| pl | Pierwotny skórny chłoniak rozlany z dużych komórek B, typu nogi | PCDLBCL, LT |
| cs | Primární kožní difúzní velkobuněčný B-lymfom dolních končetin | = en |
| uk | Первинна шкірна дифузна велика В-клітинна лімфома, ножний тип | = en |
| zh | 原发性皮肤弥漫性大B细胞淋巴瘤腿型 | = en |
| tr | Primer kütanöz diffüz büyük B hücreli lenfoma, bacak tipi | PCDLBCL, LT |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | NTBT | attributed | validated |
| ICD-11 | 2A81.A | E | specific | validated |
| MONDO | 0006383 | E | validated | |
| UMLS | C1709656 | 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:178554Aggressive primary cutaneous B-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/178544
- This record as JSON: /data/d/178544.json