Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease
| Autor: | Victoria Campbell, Kevin Marriott, Rex Stanbridge, Abdul Shlebak |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2015 |
| Předmět: | |
| Zdroj: | Case Reports in Hematology, Vol 2015 (2015) |
| Druh dokumentu: | article |
| ISSN: | 2090-6560 2090-6579 |
| DOI: | 10.1155/2015/703803 |
| Popis: | von Willebrand disease type 3 (VWD3) is a rare but the most severe form of von Willebrand disease; it is due to almost complete lack of von Willebrand factor activity (VWF:RCo). It is inherited as autosomal recessive trait; whilst heterozygote carriers have mild, or no symptoms, patients with VWD3 show severe bleeding symptoms. In the laboratory, this is characterised by undetectable VWF:Ag, VWF:RCo, and reduced levels of factor VIII < 0.02 IU/dL. The bleeding is managed with von Willebrand/FVIII factor concentrate replacement therapy. In this rare but challenging case we report on the successful excision and repair of an ascending aortic aneurysm following adequate VWF/FVIII factor concentrate replacement using Haemate-P. |
| Databáze: | Directory of Open Access Journals |
| Externí odkaz: |
|