Enteropathy-associated T-cell lymphoma
Synonyms: EATL · ETTL · Enteropathy-associated T-cell lymphoma type 1 · Enteropathy-type T-cell lymphoma · Intestinal T-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare T-cell non-Hodgkin lymphoma characterized by a neoplasm of intraepithelial T-cells mostly occurring in the jejunum or ileum in patients with celiac disease. The lesion may be multifocal and form ulcerating nodules, plaques, strictures, or an exophytic mass. The mesentery and mesenteric lymph nodes are commonly involved. Patients typically present with abdominal pain, malabsorption or diarrhea, anorexia, weight loss, fatigue, nausea, vomiting, and sometimes intestinal perforation or hemorrhage. Prognosis is generally poor.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Enteropathy-associated T-cell lymphoma | EATL · ETTL · Enteropathy-associated T-cell lymphoma type 1 · Enteropathy-type T-cell lymphoma · Intestinal T-cell lymphoma |
| fr | Lymphome T associé à une entéropathie | EATL · ETTL · Lymphome T associé à une entéropathie de type 1 · Lymphome T intestinal · Lymphome T type entéropathie |
| de | Enteropathie-assoziiertes T-Zell-Lymphom | EATL · ETTL · Enteropathie-assoziiertes T-Zell-Lymphom Typ 1 · T-Zell-Lymphom vom Enteropathie-Typ · T-Zell-Lymphom, intestinales |
| es | Linfoma de células T asociado a enteropatía | EATL · ETTL · Linfoma de células T asociado a enteropatía tipo 1 · Linfoma de células T tipo enteroptía · Linfoma intestinal de células T |
| it | Linfoma a cellule T associato a enteropatia | EATL · ETTL · Linfoma a cellule T-enteropatia · Linfoma intestinale a cellule T · Linfoma tipo 1 a cellule T associato a enteropatia |
| nl | Enteropathie-geassocieerd T-cellymfoom | EATL · ETTL · Enteropathie-geassocieerd T-cellymfoom type 1 · Enteropathie-type T-cellymfoom · Intestinaal T-cellymfoom |
| pt | Linfoma de células T associado a enteropatia | EATL · ETTL · Linfoma de células T associado a enteropatia tipo 1 · Linfoma de células T intestinal · Linfoma de células T, tipo enteropático |
| pl | Chłoniak T-komórkowy enteropatyczny | EATL · Enteropathy-type T-cell lymphoma · ETTL · Jelitowy chłoniak z komórek T · Chłoniak z komórek T związany z enteropatią |
| cs | T-buněčný lymfom asociovaný s enteropatií | EATL · ETTL · T-buněčný lymfom typu enteropatie · Intestinální T-buněčný lymfom |
| uk | Т-клітинна лімфома, асоційована з ентеропатією | EATL · Т-клітинна лімфома, асоційована з ентеропатією, тип 1 · Т-клітинна лімфома типу ентеропатії · ETTL · Кишкова Т-клітинна лімфома |
| zh | 肠病相关T细胞淋巴瘤 | 肠病型T-细胞淋巴瘤 |
| tr | Enteropati-ilişkili T-hücreli lenfoma | EATL · ETTL · Bağırsak T hücreli lenfoma · Enteropati tipi T hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.2 | E | specific | validated |
| ICD-11 | 2A90.7 | E | specific | validated |
| MONDO | 0019473 | E | validated | |
| MeSH | D058527 | E | validated | |
| UMLS | C0456889 | E | validated | |
| GARD | 9809 | E | validated | |
| MedDRA | 10073481 | 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:423793Rare tumor of small intestine
rare gastroenterological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:171918T-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
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 / 1 000 000 | 0.0111 / 100 000 | United States | Value and class | Validated | 26104061[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.1 / 100 000 | Netherlands | Value and class | Validated | 18618372[PMID] |
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/86880
- This record as JSON: /data/d/86880.json