Therapies that can be used to treat VWD and AVWS

• Intravenous: 0.3 mcg/kg (maximum dose, 20 mcg) in 50 mL saline over 20 minutes

or

• Nasal spray: Weight >50 kg: 300 mcg (1 spray in each nostril); weight <50 kg: 150 mcg (1 spray in 1 nostril)

• Adequate response to a test dose is ideally established before use, but a patient with AVWS and acute bleeding may receive the first dose as a therapeutic trial
• Use caution when an antifibrinolytic agent is given concurrently due to risk of thrombosis
• Dose may be repeated after 12 hours and 24 hours
• Tachyphylaxis and hyponatremia may occur; monitor hemostasis and serum sodium
• Thrombocytopenia may worsen in some type 2B patients

• Major bleeding or surgery: Initial dose 40 to 60 ristocetin cofactor units/kg followed by 20 to 40 ristocetin cofactor units/kg every 12 to 24 hours to keep VWF level 50 to 100 international units/dL for 7 to 14 days, or as indicated clinically
• Minor bleeding or surgery: Initial dose 30 to 60 ristocetin cofactor units/kg followed by 20 to 40 ristocetin cofactor units/kg every 12 to 48 hours to keep VWF level >30 international units/dL for 3 to 5 days, or less as indicated clinically

• Dose and duration based on clinical experience
• Case reports have described the use of continuous infusion (2 to 15 international units/kg per hour) in cases of serious bleeding that does not respond to intermittent dosing
• Increased doses or more frequent administration may be necessary in AVWS

• Major bleeding or surgery: Initial dose 50 to 80 international units/kg, followed by 40 to 60 international units/kg every 8 to 24 hours to keep the VWF level 50 to 100 international units/kg for 2 to 3 days or longer, as needed clinically
• Minor bleeding or surgery: Initial dose 40 to 50 international units/kg, followed by 40 to 50 international units/kg every 8 to 24 hours as needed clinically

• In patients with less than 40% factor VIII activity, 1 dose of recombinant factor VIII is given (dose ratio of 1 to 1.3 for rFVIII to rVWF) within 10 minutes of the first dose of rVWF
• Published studies using rVWF are limited, and more data are needed to assess responses in patients of differing ages and severities of VWD and in specific clinical settings

• Aminocaproic acid, 25 to 50 mg/kg per dose orally (maximum 5 g dose) 4 times per day

or

• Tranexamic acid, 25 mg/kg per dose orally every 6 to 8 hours or 10 mg/kg intravenously 3 times per day

• Can be used alone or in conjunction with other therapies; use caution when combined with DDAVP
• Especially useful for mucosal bleeding (often used for dental procedures)
• Dose reduction may be required in patients with impaired kidney function

• 1 g/kg intravenously once daily for 2 days*

• May be particularly helpful in monoclonal gammopathies
• May be used in conjunction with VWF concentrates and/or DDAVP, particularly when treating AVWS associated with autoimmune disease