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...

Full description

Saved in:
Bibliographic Details
Published in:Case reports in hematology 2015-01, Vol.2015 (2015), p.1-8
Main Authors: Shlebak, Abdul, Stanbridge, Rex, Marriott, Kevin, Campbell, Victoria
Format: Article
Language:English
Subjects:
Blood platelets
Case Report
Clinical medicine
Cysts
Drug therapy
Molecular weight
Postoperative period
Risk factors
Studies
Surgery
Thrombosis
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2090-6560
2090-6579
DOI:10.1155/2015/703803