NK-cell enteropathy
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Natural killer (NK)-cell enteropathy is a benign NK-cell lymphoproliferative disease characterized by minor abdominal symptoms (abdominal pain, diverticulosis, constipation and reflux) due to NK cell-derived lesions in the mucosal layer of the gastrointestinal tract and often mistaken for NK or T-cell lymphoma.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | NK-cell enteropathy | |
| fr | Entéropathie à cellules NK | |
| de | NK-Zellen-Enteropathie | |
| es | Enteropatía de células NK | |
| it | Enteropatia a cellule NK | |
| nl | NK-cel-enteropathie | |
| pt | Enteropatia de células NK | |
| pl | Enteropatia komórek NK | |
| cs | Enteropatie NK-buněk | |
| uk | NK-клітинна ентеропатія | |
| zh | NK细胞肠病 | |
| tr | NK-hücresi enteropatisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K52.8 | NTBT | attributed | validated |
| ICD-11 | DA94.Y | NTBT | attributed | validated |
| MONDO | 0016996 | E | validated | |
| UMLS | C4509932 | 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:117569Rare intestinal disease
rare gastroenterological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 8 cases | Worldwide | Case(s) | Validated | 20966166[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
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
14 annotations · validated 2017-06-14| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000969 | Edema | |
| HP:0100828 | Increased T cell count | |
| HP:0005523 | Lymphoproliferative disorder | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| Occasional (29-5%) | ||
| HP:0004295 | Abnormality of the gastric mucosa | |
| HP:0002253 | Colonic diverticula | |
| HP:0002019 | Constipation | |
| HP:0002014 | Diarrhea | |
| HP:0002588 | Duodenal ulcer | |
| HP:0002592 | Gastric ulcer | |
| HP:0002020 | Gastroesophageal reflux | |
| HP:0002573 | Hematochezia | |
| HP:0005266 | Intestinal polyp | |
| HP:0012425 | Stercoral ulcer | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 20966166[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/263665
- This record as JSON: /data/d/263665.json