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Related Experiment Video

Updated: Jun 4, 2026

Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation

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.

Paula D James¹, Anne C Goodeve

¹Queen's University, Kingston, Ontario, Canada. jamesp@queensu.ca

Genetics in Medicine : Official Journal of the American College of Medical Genetics

|February 4, 2011

Summary

This summary is machine-generated.

Von Willebrand disease is an inherited bleeding disorder causing excessive bleeding. Diagnosis involves specialized tests, and treatments include clotting factor concentrates or desmopressin.

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Area of Science:

Hematology
Genetics

Background:

Von Willebrand disease (VWD) is a prevalent inherited bleeding disorder.
It is characterized by excessive mucocutaneous bleeding, including epistaxis, bruising, and menorrhagia.

Purpose of the Study:

To outline the subtypes, diagnosis, and management of von Willebrand disease.

Main Methods:

Diagnosis relies on specialized assays of von Willebrand factor (VWF) and molecular genetic testing.
Subtypes include quantitative (1 and 3) and qualitative variants (2A, 2B, 2M, 2N).

Main Results:

Type 2 variants differ in VWF-dependent platelet adhesion and VWF multimer composition.
Type 2N involves reduced binding of VWF to factor VIII.

Conclusions:

Severe bleeding is managed with VWF/factor VIII concentrates.
Mild bleeding may respond to desmopressin, fibrinolytic inhibitors, or hormones.