Adult T-cell leukemia/lymphoma
Synonyms: ATLL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
A rare, virus associated tumor due to human T-cell leukemia virus type 1 or human T-cell lymphotropic virus type 1 (HTLV-1) and is characterized by the presence of anti-HTLV-1 antibodies, and malignant, mature, medium-sized T cells with condensed chromatin and polylobated nuclei. The malignant cells exhibit a mature CD4+ T cells phenotype and express CD2, CD5, CD25, CD45RO, HLA-DR, and T-cell receptor αβ. Presentation is heterogeneous and is typically of aggressive leukemia or lymphoma, variable skin eruptions, and visceral organ involvement.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Adult T-cell leukemia/lymphoma | ATLL |
| fr | Leucémie/lymphome T de l'adulte | = en |
| de | Adulte T-Zell-Leukämie/Lymphom | = en |
| es | Leucemia/linfoma de células T del adulto | = en |
| it | Leucemia/linfoma dell'adulto a cellule T | = en |
| nl | Volwassen T-celleukemie/lymfoom | = en |
| pt | Leucemia ou linfoma das células T do adulto | |
| pl | Białaczka/chłoniak dorosłych z komórek T | = en |
| cs | Adultní T-buněčný lymfom/leukémie | = en |
| uk | Т-клітинна лейкемія/лімфома дорослих | = en |
| zh | 成人T细胞白血病/淋巴瘤 | = en |
| tr | Yetişkin T-hücreli lösemi / lenfoma | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C91.5 | NTBT | attributed | validated |
| ICD-11 | 2A90.5 | E | specific | validated |
| MONDO | 0019471 | E | validated | |
| MeSH | D015459 | E | validated | |
| UMLS | C0023493 | E | validated | |
| MedDRA | 10001413 | 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:178551Aggressive primary cutaneous T-cell lymphoma
rare skin diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:289635Rare virus associated tumor
rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 3 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2014[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/86875
- This record as JSON: /data/d/86875.json