Management of Bleeds or Invasive Procedures with FVIII/VWF or VWF Concentrates
Recommendations on the management of acute bleeds or invasive procedures with FVIII/VWF or VWF concentrates
| Product | Type of bleed or surgery | Loading dose IU VWF:RCo/kg BW | Maintenance dose IU VWF:RCo/kg BW | Therapeutic goal VWF and FVIII trough activity levels |
|---|---|---|---|---|
| Haemate* FVIII/VWF 1:2 |
Minor/moderate | 40-50 IU/kg | 40-50 IU/kg 12-24 h until bleed controlled | > 30-50 IU/dL |
| Major | 60-80 IU/kg | 40-50 IU/kg every 8-12 h for at least 3 days Then 40-60 IU/kg daily for at least 7 days |
> 50 IU/dL then > 30 IU/dL |
|
| Wilate* FVIII/VWF 1:1 |
Minor/moderate | 20-40 IU/kg | 20-30 IU/kg 12-24 h until bleed controlled | > 30-50 IU/dL |
| Major | 40-60 IU/kg | 40-60 IU/kg 12-24 h for at least 3 days Then 20-40 IU/kg daily for at least 5-7 days |
> 50 IU/dL then > 30 IU/dL |
|
| Wilfactin** FVIII/VWF 0:1 |
Minor/moderate | 40-50 IU/kg | 40-50 IU/kg 12-24 h until bleed controlled | > 30-50 IU/dL |
| Major | 40-80 IU/kg | 40-60 IU/kg 12-24 h for at least 3 days Then 20-40 IU/kg daily for at least 7 days |
> 50 IU/dl then > 30 IU/dL |
|
| Veyondi** FVIII/VWF 0:1 |
Minor/moderate | 40-50 IU/kg | 40–50 IU/kg 12–24 h until bleed controlled | > 30-50 IU/dL |
| Major | 50-80 IU/kg | 40-60 IU/kg 8-24h for 2-3 days Then 20-40 IU/kg daily for at least 7 days |
> 50 IU/dL then > 30 IU/dL |
For levels with repeat dosing, adjust dose on VWF/FVIII activity levels.
* Continued treatment using a FVIII-containing VWF product may cause an excessive rise in FVIII activity, which may increase the risk of thromboembolic events.
In Wilate, each unit of VWF activity comes with more FVIII activity than in Haemate and there is need for fewer VWF units to get both VWF and FVIII targets into range.
** First dose should be started on the preoperative day or rFVIII should also be administered if patient FVIII < 40 IU/dL. A concentrate devoid of FVIII is suitable for patients having risk of thrombosis.
Minor/moderate bleeds: nose, mouth, mucocutaneous, muscle, soft tissue, heavy menstrual bleeds.
Major bleeds: severe or refractory epistaxis, CNS trauma, hemarthrosis, traumatic hemorrhage.
Dosage depends also on the VWD type and baseline VWF/FVIII activity levels: type 1 moderate or severe (VWF:Act typically <30 IU/dL), type 2 and type 3 VWD.
Preoperative target plasma VWF/FVIII activity levels >80-100 IU/dL in major surgery and >50 IU/dL in minor surgery.
Prophylactic maintenance treatment may be required up to 3-6 months in GI bleeds.
Recommendations on the management of acute bleeds and therapeutic targets
| Management of specific bleeds | Patient’s body weight (kg)* |
|---|---|
| Loading dose (IU/kg) | < 20 | 20-40 | 40-60 | 60-80 | 80-95 | 95-115 | |
| Minor bleed (nose, mouth, mucocutaneous | 30-50 | 500 | 500-2000 | 1000-3000 | 2000-4000 | 2500-4500 | 3000-5500 |
| Muscle, soft tissue | 30-50 | 500 | 500-2000 | 1000-3000 | 2000-4000 | 2500-4500 | 3000-5500 |
| Menorrhagia | 20-40 | - | 500-1000 | 1000-2000 | 2000-2500 | 2500-3000 | 2500-4000 |
| GI-bleed | 40-60 | 500 | 1000-2000 | 1500-3000 | 2500-3500 | 3500-5500 | 4000-6500 |
| Major bleed (severe or refractory epistaxis, CNS trauma, hearmathrosis, traumatic hemorrhage) | 40-60 | 1000 | 1500-3000 | 2500-4500 | 3500-6000 | 4500-7000 | 5000-8000 |
In addition to VWF/FVIII or VWF concentrated, the patient is given tranexamic acid.
* Dosage depends also on the VWD type and baseline VWF/FVIII activity levels.
Treatment is continued until the bleeding stops, for several days in major bleeds.
Prophylactic maintenance treatment may be required up to 3-6 months in GI bleeds.
Recommendations on the management of surgeries and other invasive procedures for patients with VWD
| Surgery and other invasive procedures | Patient’s body weight (kg)* |
|
Loading dose (IU/kg) |
<15 |
15-25 |
25-35 |
35-45 |
45-55 |
55-65 |
65-75 |
75-85 |
85-95 |
95-105 |
|
|
Major surgery VWF:Act >50 IU/dL or higher |
50-80 |
500-1000 |
500-2000 |
1000-2500 |
2000-3500 |
2000-4500 |
2500-5000 |
3000-6000 |
3500-6500 |
4000-7000 |
5000-8000 |
|
Maintenance dose every 12-24 h |
20-40 |
500 |
500-1000 |
500-1500 |
1000-1500 |
1000-2000 |
1500-2500 |
1500-3000 |
2000-3500 |
2000-4000 |
2500-4000 |
|
Minor surgery VWF:Act >30-50 IU/dL |
30-50 |
500 |
500-1000 |
500-1500 |
1000-2000 |
1500-2500 |
1500-3000 |
2000-3500 |
2000-4000 |
2500-4500 |
3000-5000 |
|
Maintenance dose every 12-24 h |
20-30 |
500 |
500 |
500-1000 |
1000-1500 |
1500 |
1500-2000 |
2000 |
2000-2500 |
2000-3000 |
2500-3000 |
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* Dosage depends also on the VWD type and baseline VWF/FVIII activity levels.
The initial dose is given 30-60 minutes before the procedure.
The duration of treatment is determined by the VWD type and the nature of the invasive procedure by closely monitoring the patient’s clinical and laboratory status.
In case of major surgery, in patients with severe VWD, treatment should be given for at least 1-2 weeks post surgery.
Key Adjustments by Product
Haemate®: Higher VWF:FVIII ratio (~2.4:1), often need more IU/kg VWF, but FVIII rises slower.
Wilate®: Balanced 1:1 ratio, lower IU/kg doses suffice, monitor FVIII closely (risk of overshoot).
Wilfactin® / Veyvondi® (VWF-only): Always consider adding FVIII with first dose if baseline FVIII < 40 IU/dL. Advantage: less FVIII accumulation long-term.