Key Points
- To review the pathophysiological mechanisms, classification schemes, genetic foundations, and therapeutic approaches for von Willebrand disease and acquired von Willebrand syndrome.
- Synthesis of phenotypic, biochemical, and multimer electrophoretic classification standards for von Willebrand factor defects.
- Review of genotype-phenotype correlations, international registry findings, and consensus treatment guidelines.
- Von Willebrand factor drives primary hemostasis through shear-dependent platelet adhesion and stabilizes coagulation factor VIII in circulation.
- Quantitative and qualitative defects are differentiated via functional assays, multimer electrophoresis, and molecular genetic testing.
- Desmopressin corrects hemostatic defects in approximately 70% of patients (primarily type 1 vWD), while the remaining patients require von Willebrand factor concentrates.
Structured PICO
PPopulationPatients with von Willebrand disease (vWD) or acquired von Willebrand syndrome (avWS)
IInterventionDesmopressin or vWF concentrates
This review summarizes the pathophysiology, classification, and treatment of von Willebrand disease, highlighting desmopressin as the primary treatment for type 1 vWD.