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Updated: May 13, 2026
08:30
Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
Published on: August 14, 2017
Von Willebrand disease: pathogenesis and management
1Department of Cell Therapy and Hematology, San Bortolo Hospital, Vicenza, Italy. rodeghiero@hemato.ven.it
Thrombosis Research
|March 5, 2013
Summary
Diagnosing von Willebrand disease (VWD) involves a bleeding score and key tests like VWF activity and antigen. Desmopressin is a primary treatment, with factor replacement for specific cases.
Area of Science:
- Hematology
- Genetics
- Internal Medicine
Background:
- Recent studies propose a unifying pathophysiological concept for von Willebrand factor (VWF) defects.
- Simple VWF-related activities are crucial for diagnosing von Willebrand disease (VWD).
Purpose of the Study:
- To highlight the importance of standardized bleeding history and laboratory tests in VWD diagnosis.
- To outline diagnostic and therapeutic strategies for VWD management.
Main Methods:
- Utilizing a standardized bleeding history (bleeding score).
- Employing laboratory tests: VWF ristocetin cofactor activity, VWF antigen, and factor VIII (FVIII).
- Assessing ristocetin-induced platelet aggregation (RIPA).
Main Results:
- Bleeding score and VWF-related laboratory tests are critical for VWD diagnosis.
- Desmopressin is the first-choice treatment for responsive patients.
- VWF/FVIII concentrates are used for unresponsive patients or complex cases.
Conclusions:
- A unified approach using bleeding history and VWF assays aids VWD diagnosis.
- Desmopressin and VWF/FVIII concentrates are effective therapeutic options.
- Specific considerations are needed for menorrhagia and parturition in VWD patients.
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