Prophylactic treatment with VWF concentrates

Long-term prophylaxis with a VWF concentrate should be tailored in VWD patients with a history of severe and frequent bleeds. Intracranial bleed and joint bleeds in type 3 VWD patients are the strongest indication for further prophylactic treatment. If nose bleeds, menorrhagia or gastrointestinal bleeds are severe and cause anemia despite iron supplementation and a major impact on social life, and/or other treatment modalities have failed, prophylactic replacement therapy should be provided. An international multicenter cohort study has shown that prophylactic treatment of VWD is efficacious.

When used for prophylaxis in outpatients, a VWF concentrate at a dose of about 20-50 IU VWF:RCo/kg i.v. administered 2-3 times per week may be sufficient to prevent bleeds. Levels of VWF:RCo and FVIII:C should be monitored along the use of prophylaxis and in association of patient visits or invasive procedures.