ORPHA:98827
Unclassified myelodysplastic syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Unclassified myelodysplastic syndrome (MDS-U) is a subtype of myelodysplastic syndrome (MDS) with atypical features of uncertain clinical significance.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Unclassified myelodysplastic syndrome | |
| fr | Syndrome myélodysplasique non-classifié | |
| de | Myelodysplastisches Syndrom, nicht-klassifizierbares | |
| es | Síndrome mielodisplásico no clasificado | |
| it | Sindrome mielodisplastica non classificata | |
| nl | Niet-geclassificeerd myelodysplastisch syndroom | |
| pt | Síndrome mielodisplásico, não classificado | |
| pl | Niesklasyfikowany zespół mielodysplastyczny | |
| cs | Neklasifikovaný myelodysplastický syndrom | |
| uk | Некласифікований мієлодиспластичний синдром | |
| zh | 未分类骨髓增生异常综合征 | |
| tr | Sınıflandırılmamış miyelodisplastik sendrom |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D46.7 | NTBT | attributed | validated |
| ICD-11 | 2A37 | E | specific | validated |
| MONDO | 0020315 | E | validated | |
| UMLS | C5681636 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:86836Refractory cytopenia with multilineage dysplasia
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | Unknown | — | Worldwide | Class only | Validated | WHO Tumour classification[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | WHO Tumour classification[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.
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HPO phenotypes
10 annotations · validated 2019-06-11| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002863 | Myelodysplasia | |
| Frequent (79-30%) | ||
| HP:0045040 | Abnormal lactate dehydrogenase activity | |
| HP:0001871 | Abnormality of blood and blood-forming tissues | |
| HP:0012378 | Fatigue | |
| HP:0012148 | Multiple lineage myelodysplasia | |
| Occasional (29-5%) | ||
| HP:0002960 | Autoimmunity | |
| HP:0005528 | Bone marrow hypocellularity | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0030166 | Night sweats | |
| Very rare (<4-1%) | ||
| HP:0004808 | Acute myeloid leukemia | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 26799610[PMID]_28297619[PMID]_27039723[PMID]_17709138[PMID]_30091023[PMID]_30555034[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/98827
- This record as JSON: /data/d/98827.json