von Willebrand factor
Supports platelet adhesion and stabilization of factor VIII.
von Willebrand disease
The most common congenital clotting disorder, with very different forms of manifestation and a need for accurate diagnosis.
Variable symptoms
Manifestations may be mild, moderate or severe, depending on the disease type.
Diagnosis
Confirmation is done through clotting tests and von Willebrand factor measurement.
von Willebrand disease is the most common congenital coagulopathy, transmitted in an autosomal dominant pattern. It is characterized by qualitative or quantitative deficiencies of von Willebrand factor, a glycoprotein essential for platelet adhesion and factor VIII stabilization.
Disease severity
Symptoms vary from minor bleeding to severe hemorrhage; severity differs between families and even within the same family.
Disease diagnosis
Diagnosis is made through clotting tests and von Willebrand factor measurement. Bleeding time, Quick and TPT tests, as well as factor VIII and IX measurements can guide the diagnosis toward hemophilia or von Willebrand disease.
Treatment
Treatment includes replacement therapy and preventive measures, such as avoiding anticoagulant medicines and correctly monitoring bleeding episodes.