| verworven ziekte van von Willebrand (aandoening) | | verworven ziekte van von Willebrand | | Acquired von Willebrand disease | | Acquired von Willebrand's disease
Acquired von Willebrand syndrome
| | Acquired von Willebrand disease (VWD) is a rare bleeding disorder marked by the same biological anomalies as those seen in hereditary von Willebrand disease (VWD) but which occurs in association with another underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies. There are three principle pathogenic mechanisms described which are firstly the presence of autoantibodies (inhibiting or noninhibiting) that form immune complexes with the von Willebrand factor (VWF) leading to rapid clearance of VWF from the circulation. Secondly, absorption of VWF onto malignant cell clones and thirdly the increased proteolysis of high molecular weight VWF multimers under abnormal blood conditions caused by cardiovascular malformations (such as aortic valve stenosis). |
| | DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | D68.4 | | Term | Verworven stollingsfactordeficiëntie |
|
| SNOMED CT to Orphanet simple map | 99147 |
| SNOMED CT to ICD-10 extended map | | Target | D68.0 | | Rule | TRUE | | Advice | ALWAYS D68.0 | | Correlation | SNOMED CT source code to target map code correlation not specified |
|
|
