Acquired von Willebrand syndrome
Synonyms: Acquired von Willebrand disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare bleeding disorder characterized by defects in von Willebrand factor, similar to those seen in hereditary von Willebrand disease (VWD), but which are caused by an underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acquired von Willebrand syndrome | Acquired von Willebrand disease |
| fr | Syndrome de von Willebrand acquis | Maladie de von Willebrand acquise |
| de | Von-Willebrand-Syndrom, erworbenes | AWVS |
| es | Síndrome de Von Willebrand adquirido | |
| it | Malattia di von Willebrand acquisita | |
| nl | Verworven syndroom van von Willebrand | Verworven ziekte van Von Willebrand |
| pt | Doença de von Willebrand adquirida | |
| pl | Nabyty zespół von Willebranda | Nabyta choroba von Willebranda |
| cs | Získaný von Willebrandův syndrom | Získaná von Willebrandova choroba |
| uk | Набутий синдром фон Віллебранда | Набута хвороба фон Віллебранда |
| zh | 获得性假血友病综合征 | 获得性血管性血友病 |
| tr | Edinsel von Willebrand sendromu | Edinsel Von Willebrand hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D68.4 | NTBT | attributed | validated |
| ICD-11 | 3B2Y | NTBT | index | validated |
| MONDO | 0020460 | E | validated | |
| UMLS | C0272362 | E | validated | |
| GARD | 5573 | E | validated | |
| MedDRA | 10069495 | 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:166775Rare hemorrhagic disorder due to an acquired coagulation factor defect
rare hematological 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 | 300 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| 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
27 annotations · validated 2019-10-08| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0030680 | Abnormal cardiovascular system morphology | |
| HP:0004377 | Hematological neoplasm | |
| HP:0030129 | Impaired ristocetin cofactor assay activity | |
| HP:0008151 | Prolonged prothrombin time | |
| HP:0003125 | Reduced factor VIII activity | |
| HP:0008330 | Reduced von Willebrand factor activity | |
| Occasional (29-5%) | ||
| HP:0001659 | Aortic regurgitation | |
| HP:0001650 | Aortic valve stenosis | |
| HP:0025406 | Asthenia | |
| HP:0000978 | Bruising susceptibility | |
| HP:0000421 | Epistaxis | |
| HP:0000471 | Gastrointestinal angiodysplasia | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0000790 | Hematuria | |
| HP:0001931 | Hypochromic anemia | |
| HP:0002615 | Hypotension | |
| HP:0005261 | Joint hemorrhage | |
| HP:0002249 | Melena | |
| HP:0000132 | Menorrhagia | |
| HP:0100608 | Metrorrhagia | |
| HP:0001653 | Mitral regurgitation | |
| HP:0001897 | Normocytic anemia | |
| HP:0001934 | Persistent bleeding after trauma | |
| HP:0001642 | Pulmonic stenosis | |
| HP:0005505 | Refractory anemia | |
| HP:0001933 | Subcutaneous hemorrhage | |
| Very rare (<4-1%) | ||
| HP:0002170 | Intracranial hemorrhage | |
Annotation source: 29239101[PMID]_30057374[PMID]_30214173[PMID]_31004311[PMID]_31293998[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99147
- This record as JSON: /data/d/99147.json