Primary cutaneous follicle center lymphoma
Synonyms: PCFCL
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare, indolent primary cutaneous B-cell lymphoma characterized by a solitary or grouped erythematous plaques or tumors, preferentially located on the head, neck or trunk region, and composed of centroblasts and centrocytes arranged in a follicular, diffuse, or mixed growth pattern. The lesions are smooth and typically do not ulcerate. The neoplastic cells express pan B cell markers and Bcl-6, and typically lack Bcl-2.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous follicle center lymphoma | PCFCL |
| fr | Lymphomes B centrofolliculaire cutané primitif | |
| de | Lymphom, primär kutanes, follikuläres | = en |
| es | Linfoma cutáneo primario del centro folicular | = en |
| it | Linfoma cutaneo primitivo del centro follicolare | |
| nl | Primair cutaan follikelcenterlymfoom | = en |
| pt | Linfoma primário cutâneo do centro folicular | |
| pl | Pierwotny skórny chłoniak grudkowy | = en |
| cs | Primární kožní B-lymfom z folikulárních center | = en |
| uk | Первинна шкірна лімфома фолікулярного центру | = en |
| zh | 原发性皮肤滤泡中心性淋巴瘤 | = en |
| tr | Primer kutanöz folikül santral lenfoma | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C82.6 | E | specific | validated |
| ICD-11 | 2A80.3 | E | specific | validated |
| MONDO | 0015814 | E | validated | |
| UMLS | C1333171 | E | validated | |
| GARD | 13701 | 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/178540
- This record as JSON: /data/d/178540.json