OBJECTIVES: Von Willebrand Disease (VWD) is the most common inherited bleeding disorder, caused by a deficiency or abnormality of the Von Willebrand factor (VWF). Long-term prophylaxis (LTP) with Factor VIII-containing VWF product (FVIII/VWF) is used for patients with severe VWD. However, FVIII/VWF is not enough effective in some cases. Furthermore repeated infusions of FVIII/VWF may increase the risk of thromboembolic events. The objective of the analysis was to assess the economic impact of VWF concentrate almost devoid of FVIII as an alternative to FVIII/VWF for a patient on LTP with recurrent bleedings. METHODS: A cost-consequences analysis was adopted to assess the economic impact from the NHS and Society perspectives. The cost analysis was based on one patient case (type 1 VWD, recurrent gum bleedings), treated with FVIII/VWF and then with VWF concentrate almost devoid of FVIII. The costs included direct costs (drug acquisition, hospital admissions, outpatient visits, red blood cells units) and indirect costs (working days lost). Data were gathered from a questionnaire (Hemophilia Reference Center of Catania). The health care costs were calculated by DRG analysis that assessed DRG refund value for day hospital and standard hospitalization. The indirect costs were calculated on the basis of the annual income average. RESULTS: The analysis showed a reduction of the number of bleedings when treating with VWF concentrate almost devoid of FVIII (30 vs 0), minimizing the cost per bleeding episodes (€87,957 vs €0), hospitalizations (€3,252 vs 0), monitoring visits (€331 vs €165) and the number of working days lost (26 vs 2). The annual health care costs and indirect costs avoided were €49,684 and €3,734 respectively. CONCLUSIONS: The replacement therapy with VWF concentrate almost devoid of FVIII decreased the consumption of hospital resources and reduced the number of working days lost per bleedings together with the discomfort related to bleedings.