Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease
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 s...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2015/703803 |
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| _version_ | 1832555087321366528 |
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| author | Victoria Campbell Kevin Marriott Rex Stanbridge Abdul Shlebak |
| author_facet | Victoria Campbell Kevin Marriott Rex Stanbridge Abdul Shlebak |
| author_sort | Victoria Campbell |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-f55b24a728344487afe292b22c8f19d0 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-f55b24a728344487afe292b22c8f19d02025-02-03T05:49:37ZengWileyCase Reports in Hematology2090-65602090-65792015-01-01201510.1155/2015/703803703803Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) DiseaseVictoria Campbell0Kevin Marriott1Rex Stanbridge2Abdul Shlebak3Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UKDepartment of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UKDepartment of Cardiothoracic Surgery, Imperial College Healthcare NHS Trust, London W2 1NY, UKDepartment of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UKvon 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.http://dx.doi.org/10.1155/2015/703803 |
| spellingShingle | Victoria Campbell Kevin Marriott Rex Stanbridge Abdul Shlebak Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease Case Reports in Hematology |
| title | Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease |
| title_full | Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease |
| title_fullStr | Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease |
| title_full_unstemmed | Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease |
| title_short | Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease |
| title_sort | successful aortic aneurysm repair in a woman with severe von willebrand type 3 disease |
| url | http://dx.doi.org/10.1155/2015/703803 |
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