Abstract
Type 3 von Willebrand disease (VWD) is the most severe form of this bleeding disorder owing to a virtually complete deficiency of von Willebrand factor (VWF). Type 3 VWD is inherited as a recessive trait-heterozygous relatives have mild or no bleeding symptoms, while most patients with type 3 VWD show severe bleeding because they are characterized by undetectable levels of VWF antigen and reduced concentrations (<0.10 IU/dL) of factor VIII. Although rare, type 3 VWD is of major interest because of its severe clinical presentation, the need for replacement therapy, and the risk of occurrence of alloantibodies after the infusion of plasma-derived VWF concentrates. The molecular basis of type 3 VWD has been studied in detail and numerous molecular defects have been identified. This chapter will focus on the clinical, laboratory, and molecular aspects of type 3 VWD that can be useful for a correct therapeutic approach in these patients.
Original language | English |
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Title of host publication | Von Willebrand Disease: Basic and Clinical Aspects |
Publisher | Wiley-Blackwell |
Pages | 148-165 |
Number of pages | 18 |
ISBN (Print) | 9781405195126 |
DOIs | |
Publication status | Published - Mar 21 2011 |
Keywords
- Alloantibodies
- Bleeding symptoms
- Laboratory tests
- Molecular and prenatal diagnosis
- Secondary long-term prophylaxis
- Surgery
- Type 3 von Willebrand disease
- Von Willebrand factor
- Von Willebrand factor concentrates
ASJC Scopus subject areas
- Medicine(all)