Table 4.

Indications for replacement therapy in VWD Reference [14].

Indications for Replacement Therapy in VWD Reference [14]

Spontaneous or post-traumatic severe bleeding Single or daily doses of 50 IU/kg of VWF to maintain FVIII:C levels >50 U/dL until bleeding stops (usually 7–10 days) * Spontaneous or post-traumatic mild to moderate bleeding Single or daily doses of 20–40 IU/kg of VWF to maintain FVIII:C levels >30 U/dL until bleeding stops (usually 1–3 days) *

Major surgery Daily doses of 50–60 IU/kg of VWF to maintain pre-operative FVIII:C and VWF:RCo levels of 80–100 U/dL until 36 h postoperatively and then >50 U/dL until healing is complete (usually 7–10 days) * Measure plasma levels of FVIII:C (and VWF:RCo ) every 12 h on the day of surgery, then ever 24 h Usual thrombo-prophylactic treatment with LMWH should be implemented in patients at high risk of venous thrombosis Minor surgery Daily or every other day doses of 30–60 IU/kg of VWF to maintain FVIII:C levels >30 U/dL until healing is complete (usually 1–5 days) * Dental extractions Single dose of 20–40 IU/kg of VWF to maintain FVIII:C levels >50 U/dL for 12 h * Delivery and puerperium Daily doses of 50 IU/kg of VWF to maintain FVIII:C levels >50 U/dL for 3–4 days

In type 3 patients with alloantibodies all plasma concentrates containing VWF must be avoided because of the risk of anaphylactic reactions. r-FVII (Recombinant FVIII) administered at very high doses by continuous intravenous infusion, or r-FVIIa can be used instead

Note: Dosing should be based on VWF:RCo content where this is available. * These dosages are indicated for VWD patients with FVIII:C/VWF:RCo levels <10 U/dL.