Anaplastic large cell lymphoma
Synonyms: ALCL · CD30 positive anaplastic large cell lymphoma · Ki-1 positive anaplastic large cell lymphoma · Primary systemic ALCL · sACL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare and aggressive peripheral T-cell non-Hodgkin lymphoma, belonging to the group of CD30-positive lymphoproliferative disorders, which affects lymph nodes and extranodal sites. It is comprised of two sub-types, based on the expression of a protein called anaplastic lymphoma kinase (ALK): ALK positive and ALK negative ALCL.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anaplastic large cell lymphoma | ALCL · CD30 positive anaplastic large cell lymphoma · Ki-1 positive anaplastic large cell lymphoma · Primary systemic ALCL · sACL |
| fr | Lymphome anaplasique à grandes cellules | ALCL · Lymphome anaplasique à grandes cellules CD30+ · Lymphome anaplasique à grandes cellules Ki-1+ · Lymphome à grandes cellules anaplasiques |
| de | Lymphom, anaplastisch großzelliges | ALCL · CD30-positives anaplastisches großzelliges Lymphom · Ki-1 positives anaplastisches großzelliges Lymphom · sACL |
| es | Linfoma anaplásico de células grandes | ALCL · ALCL sistémico primario · Linfoma anaplásico de células grandes CD30 positivo · Linfoma anaplásico de células grandes Ki-1 positivo · sACL |
| it | Linfoma anaplastico a grandi cellule | ALCL · ALCL sistemico primitivo · Linfoma anaplastico a grandi cellule CD30-positivo · Linfoma anaplastico a grandi cellule Ki-1-positivo · sACL |
| nl | Anaplastisch grootcellig lymfoom | ALCL · CD30-positief anaplastisch grootcellig lymfoom · Ki-1-positief anaplastisch grootcellig lymfoom · Primair systemisch ALCL · sACL |
| pt | Linfoma anaplásico de células grandes | |
| pl | Anaplastyczny chłoniak wielkokomórkowy | ALCL · Anaplastyczny chłoniak wielkokomórkowy CD30 pozytywny · Anaplastyczny chłoniak wielkokomórkowy Ki-1 pozytywny · Pierwotny układowy ALCL · sACL |
| cs | Anaplastický velkobuněčný lymfom | ALCL · Primární systémový ALCL · sACL · CD30 pozitivní anaplastický velkobuněčný lymfom · Ki-1 pozitivní anaplastický velkobuněčný lymfom |
| uk | Анапластична великоклітинна лімфома | ALCL · CD30-позитивна анапластична великоклітинна лімфома · Кі-1-позитивна анапластична великоклітинна лімфома · Первинна системна ALCL · sACL |
| zh | 间变性大细胞淋巴瘤 | Ki-1阳性间变性大细胞淋巴瘤 |
| tr | Anaplastik büyük hücreli lenfoma | ALCL · sACL · Primer sistemik ALCL · CD30 pozitif anaplastik büyük hücreli lenfoma · Ki-1 pozitif anaplastik büyük hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C84.6 | BTNT | specific | validated |
| ICD-10 | C84.7 | BTNT | specific | validated |
| ICD-11 | 2A90.A | BTNT | specific | validated |
| ICD-11 | 2A90.B | BTNT | specific | validated |
| MONDO | 0020325 | E | validated | |
| MeSH | D017728 | E | validated | |
| UMLS | C0206180 | E | validated | |
| GARD | 3112 | 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:171918T-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
Genes
0 direct · 1 via subtypesOrphanet records no gene directly on this code.
Recorded by Orphanet on subtypes of this disorder:
| Gene | Subtype | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|---|
| ALK | ORPHA:300895ALK-positive anaplastic large cell lymphoma | Disease-causing somatic mutation(s) in | Assessed | HGNC:427 | ENSG00000171094 | 105590 | 2p23.2-p23.1 | ALK receptor tyrosine kinase | 12112524[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 2 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2011[OTHER] |
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/98841
- This record as JSON: /data/d/98841.json