ALK-positive large B-cell lymphoma
Synonyms: ALK+ LBCL · ALK+ large B-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Status
- active
A very rare variant of diffuse large B-cell lymphoma (DLBCL) mainly affecting middle-aged immunocompetent men and characterized by a consistent primary involvement of lymph nodes (mainly in the cervical and mediastinum lymph nodes) and with infrequent extra nodal involvement of the bone marrow and other extra-nodal sites (head and neck region, liver, spleen, and gastrointestinal tract). It has an aggressive disease course, and is associated with a poor prognosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | ALK-positive large B-cell lymphoma | ALK+ LBCL · ALK+ large B-cell lymphoma |
| fr | Lymphome B à grandes cellules ALK positif | ALK+ LBCL · LBGC ALK+ · Lymphome B à grandes cellules ALK+ |
| de | ALK-positives großzelliges B-Zell-Lymphom | ALK+ LBCL · B-Zell-Lymphom, großzelliges, ALK+ |
| es | Linfoma B de células grandes ALK-positivo | ALK+ LBCL · Linfoma B de células grandes ALK+ |
| it | Linfoma a grandi cellule B positivo all'ALK | ALK+ LBCL · Linfoma a grandi cellule B ALK+ |
| nl | ALK-positief grootcellig B-cellymfoom | ALK+ LBCL · ALK+ grootcellig B-cellymfoom |
| pt | Linfoma de grandes células BALK - positivo | |
| pl | Chłoniak ALK-pozytywny z dużych komórek B | ALK+ LBCL · Chłoniak ALK+ z dużych komórek B |
| cs | ALK-pozitivní velkobuněčný B-lymfom | ALK+LBCL · ALK+ velkobuněčný B-lymfom |
| uk | ALK-позитивна велика В-клітинна лімфома | ALK+ велико-В-клітинна лімфома · ALK+ LBCL |
| zh | ALK阳性大B细胞淋巴瘤 | ALK+大B细胞淋巴瘤 |
| tr | ALK-pozitif büyük B-hücreli lenfoma | ALK+ LBCL · ALK + büyük B-hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | NTBT | index | validated |
| ICD-11 | 2A81.8 | E | specific | validated |
| ICD-11 | XH1EB9 | BTNT | specific | validated |
| MONDO | 0018225 | E | validated | |
| UMLS | C1333294 | 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:544Diffuse large B-cell lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| ALK | Part of a fusion gene in | Not yet assessed | HGNC:427 | ENSG00000171094 | 105590 | 2p23.2-p23.1 | ALK receptor tyrosine kinase |
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/364043
- This record as JSON: /data/d/364043.json