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Updated: Jan 19, 2026
Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
Published on: August 14, 2017
How I manage severe von Willebrand disease
Frank W G Leebeek1, Ferdows Atiq1
1Department of Haematology, Erasmus University Medical Centre, Rotterdam, the Netherlands.
Insights
Severe von Willebrand disease (VWD) management is challenging, especially for types 2 and 3. This review covers current and emerging treatments for severe VWD, including special circumstances like pregnancy and alloantibody development.
Area of Science:
- Hematology
- Genetics
- Internal Medicine
Background:
- Von Willebrand disease (VWD) is the most prevalent inherited bleeding disorder.
- Mild to moderate VWD cases are often managed effectively with desmopressin.
- Severe VWD, particularly types 2 and 3, presents significant management challenges.
Purpose of the Study:
- To review the current diagnostic approaches for severe VWD.
- To outline established and emerging treatment strategies for severe VWD.
- To discuss VWD management in specific complex clinical scenarios.
Main Methods:
- Comprehensive literature review of VWD diagnosis and treatment.
- Analysis of management strategies for severe VWD subtypes.
- Examination of VWD care in pregnancy, alloantibody formation, and GI bleeding.
Main Results:
- Desmopressin is effective for mild/moderate VWD but less so for severe forms.
- Management of severe VWD requires tailored approaches, often involving factor replacement.
- Special populations (pregnant, alloantibody-positive) necessitate distinct treatment plans.
Conclusions:
- Current treatments for severe VWD are evolving, with ongoing research into novel therapies.
- Personalized management is crucial for severe VWD patients, considering individual complications.
- Future treatments hold promise for improving outcomes in challenging severe VWD cases.
Abstract:
Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Most patients with mild and moderate VWD can be treated effectively with desmopressin. The management of severe VWD patients, mostly affected by type 2 and type 3 disease, can be challenging. In this article we review the current diagnosis and treatment of severe VWD patients. We will also discuss the management of severe VWD patients in specific situations, such as pregnancy, delivery, patients developing alloantibodies against von Willebrand factor and VWD patients with recurrent gastrointestinal bleeding. Moreover, we review emerging treatments that may be applied in future management of patients with severe VWD.
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