Monomorphic epitheliotropic intestinal T-cell lymphoma
Synonyms: Enteropathy-associated T-cell lymphoma type 2 · MEITL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare T-cell non-Hodgkin lymphoma characterized by monomorphic cytomorphology and epitheliotropism. It is mostly detected in the small intestine but can also be present in the colon, duodenum and stomach. It is an aggressive tumor that can disseminate to mesenteric lymph nodes, lung, liver, brain and skin. Major clinical features include abdominal pain, gastrointestinal bleeding, obstruction or perforation, diarrhoea, and weight loss. It is not associated to coeliac disease.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Monomorphic epitheliotropic intestinal T-cell lymphoma | Enteropathy-associated T-cell lymphoma type 2 · MEITL |
| fr | Lymphome T intestinal epithéliotrope monomorphe | Lymphome intestinal à cellules T de type entéropathie de type 2 · MEITL |
| de | Monomorphes epitheliotropes intestinales T-Zell-Lymphom | Enteropathie-assoziiertes T-Zell-Lymphom Typ 2 · MEITL |
| es | Linfoma intestinal de células T monomórfico epiteliotrópico | Linfoma de células T asociado a enteropatía tipo 2 · MEITL |
| it | Linfomi a cellule T del tratto gastrointestinale | Linfoma a cellule T associato ad enteropatia tipo 2 · MEITL |
| nl | Monomorf epitheliotroop intestinaal T-cellymfoom | Enteropathie-geassocieerd T-cellymfoom type 2 · MEITL |
| pt | Linfoma intestinal epiteliotrópico monomórfico de células T | Linfoma de células T associado a enteropatia tipo 2 · MEITL |
| pl | Monomorficzny epiteliotropowy chłoniak jelitowy z komórek T | = en |
| cs | Monomorfní epiteliotropní střevní T-buněčný lymfom | = en |
| uk | Мономорфна епітеліотропна кишкова Т-клітинна лімфома | Т-клітинна лімфома, асоційована з ентеропатією, тип 2 · MEITL |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.2 | NTBT | attributed | validated |
| ICD-11 | 2A90.7 | NTBT | attributed | validated |
| MONDO | 0958096 | E | validated | |
| MONDO | 958096 | E | validated | |
| UMLS | C3272525 | 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
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/652658
- This record as JSON: /data/d/652658.json