Primary cutaneous lymphoma
- Type
- Category
- Group
- Group of disorders
- Status
- active
A group of rare primary organ-specific lymphoma characterized by heterogeneous T-cell and B-cell non-Hodgkin lymphomas present in the skin. Clinical and pathological features, evolutive profile, prognosis, molecular etiology and response to therapy are highly variable between the entities belong to this group.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous lymphoma | |
| fr | Lymphome cutané primitif | |
| de | Lymphom, kutanes primäres | |
| es | Linfoma cutáneo primario | |
| it | Linfoma cutaneo primitivo | |
| nl | Primair cutaan lymfoom | |
| pt | Linfoma cutâneo primário | |
| pl | Pierwotny chłoniak skóry | |
| cs | Primární kožní lymfom | |
| uk | Первинна шкірна лімфома | |
| zh | 原发性皮肤淋巴瘤 | |
| tr | Primer kutanöz lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0018898 | E | validated | |
| MONDO | 18898 | E | validated | |
| UMLS | C1302772 | E | validated | |
| MedDRA | 10051708 | 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 (2):
- ORPHA:279911Primary organ-specific lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:79386Rare skin tumor or hamartoma
rare skin diseases; rare neoplastic diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.75 / 100 000 | Europe | Value and class | Not yet validated | 15692063[PMID]_16192622[PMID]_ ORPHANET |
| Point prevalence | 1-9 / 100 000 | — | Europe | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/542
- This record as JSON: /data/d/542.json