OBJECTIVES: To conduct a budget impact analysis of treatment and prophylaxis of acute bleedings among patients with von Willebrand disease using blood clotting factor concentrates (clotting factor VIII + von Willebrand factor) in the Russian Federation. METHODS: Clinical effectiveness assessment was based on the results of modeling of the treatment and prophylaxis of patients with von Willebrand disease using blood clotting factor concentrates (clotting factor VIII + von Willebrand factor) within one year (time horizon). The required data was obtained from literature review within PubMed and E-library, and from experts in hematological healthcare centers in Russia. Cost analysis included assessment of direct and indirect costs that can be associated with analyzed schemes of treatment (“No prophylaxis”, “Prophylaxis ¹1” – median purity concentrate VWF:RCo/FVIII=0,5/1, “Prophylaxis ¹2” - high purity concentrate VWF:RCo/FVIII=2,5/1, “Prophylaxis ¹3” - high purity concentrate VWF:RCo/FVIII=0,9/1). Cost data was based on median prices for medicines and medical services in National healthcare system in the Russian Federation. RESULTS: In case of switching from regimens: “No prophylaxis”, “Prophylaxis ¹1”, “Prophylaxis ¹2”, to the regular preventive administration with the scheme “Prophylaxis ¹3” total costs per all population within one year can be reduced by 21906000 rubles ($ 396 360), 22143000 rubles ($ 400 648) and 5752000 rubles ($ 104 075), respectively CONCLUSIONS: Obtained results demonstrates that the use of high purity concentrate VWF:RCo /FVIII=0,9/1 in treatment and prophylaxis of patients with von Willebrand disease is approved from a pharmacoeconomic point of view