Primary cutaneous anaplastic large cell lymphoma
Synonyms: Primary C-ALCL · Regressive atypical histiocytosis
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare T-cell non-Hodgkin lymphoma that affects the skin and generally shows no extracutaneous involvement at presentation. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders along with lymphomatoid papulosis with which it shares overlapping clinical and histopathologic features.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous anaplastic large cell lymphoma | Primary C-ALCL · Regressive atypical histiocytosis |
| fr | Lymphome cutané primitif anaplasique à grandes cellules | C-ALCL primaire · Histiocytose atypique régressive |
| de | Primäres kutanes anaplastisches großzelliges Lymphom | C-ALCL · Primäres C-ALCL · Regressive atypische Histiozytose |
| es | Linfoma cutáneo primario anaplásico de células grandes | C-ALCL primario · Histiocitosis atípica regresiva |
| it | Linfoma anaplastico cutaneo primitivo a grandi cellule | C-ALCL primitivo · Istiocitosi atipica regressiva |
| nl | Primair cutaan anaplastisch grootcellig lymfoom | Primair C-ALCL · Regressieve atypische histiocytose |
| pt | Linfoma anaplásico de grandes células, cutâneo primário | |
| pl | Pierwotny skórny chłoniak anaplastyczny z dużych komórek | Histiocytoza regresywna atypowa · Primary C-ALCL |
| cs | Primární velkobuněčný anaplastický kožní lymfom | Primární C-ALCL · Regresivní atypická histiocytóza |
| uk | Первинна шкірна анапластична великоклітинна лімфома | Первинний C-ALCL · Регресивний атиповий гістіоцитоз |
| zh | 原发皮肤间变性大细胞淋巴瘤 | 隐性遗传非典型性组织细胞增生 |
| tr | Primer kutanöz anaplastik büyük hücreli lenfoma | Primer C-ALCL · Gerileyen atipik histiyositoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.6 | NTBT | inclusion | validated |
| ICD-11 | 2B03.0 | E | specific | validated |
| MONDO | 0017598 | E | validated | |
| MeSH | D054446 | E | validated | |
| UMLS | C1301362 | E | validated | |
| MedDRA | 10065863 | 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:541Primary cutaneous CD30+ T-cell lymphoproliferative disease
rare skin diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| NPM1 | Part of a fusion gene in | Assessed | HGNC:7910 | ENSG00000181163 | 164040 | 5q35.1 | nucleophosmin 1 | 25349176[PMID] |
| TYK2 | Part of a fusion gene in | Assessed | HGNC:12440 | ENSG00000105397 | 176941 | 19p13.2 | tyrosine kinase 2 | 25349176[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | 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/300865
- This record as JSON: /data/d/300865.json