Primary cutaneous marginal zone B-cell lymphoma
Synonyms: PCMZL
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare, indolent primary cutaneous B-cell lymphoma characterized by multifocal, red to violaceous papules, plaques or nodules localized predominantly on the trunk and extremities. Histologically, these are dermis infiltrates consisting of small, marginal zone B cells, lymphoplasmacytic cells, and plasma cells. Marginal zone B cells express CD20, CD79a and Bcl-2, and are negative for CD5, CD10 and Bcl-6. Plasma cells are typically located at the periphery, and express CD138, CD79a, and monotypic light chains.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous marginal zone B-cell lymphoma | PCMZL |
| fr | Lymphome B cutané de la zone marginale | |
| de | Primär kutanes Marginalzonen-B-Zell-Lymphom | = en |
| es | Linfoma cutáneo primario de células B de la zona marginal | = en |
| it | Linfoma cutaneo primitivo della zona marginale a cellule B | |
| nl | Primair cutaan marginale zone B-cellymfoom | = en |
| pt | Linfoma primário cutâneo de células B da zona marginal | |
| pl | Pierwotny skórny chłoniak strefy brzeżnej | = en |
| cs | Primární kožní B-lymfom z marginální zóny | = en |
| uk | Первинна шкірна маргінальна В-клітинна лімфома | = en |
| zh | 原发性皮肤边缘区B细胞淋巴瘤 | = en |
| tr | Primer kutanöz marjinal bölge B hücreli lenfoma | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.0 | NTBT | attributed | validated |
| ICD-11 | 2A85.2 | E | specific | validated |
| MONDO | 0015813 | E | validated | |
| UMLS | C1275321 | 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:178557Indolent 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/178536
- This record as JSON: /data/d/178536.json