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Treatment of Willebrand's disease
Last reviewed: 04.07.2025
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Like any hereditary disease, von Willebrand disease cannot be cured; only treatment or prevention of the disease's manifestations are possible.
Specific hemostatic effect (von Willebrand factor concentrates, desmopressin).
Von Willebrand factor preparations are indicated for type 3 and severe other types of von Willebrand disease.
The introduction of FFP in von Willebrand disease for hemostatic purposes is not rational due to the relatively low concentration of von Willebrand factor in it. Blood coagulation factor VIII (cryoprecipitate) contains 10 times more von Willebrand factor per unit volume. Its disadvantages are a high risk of infection with blood transfusion infections and the content of a large number of ballast substances, including those with pronounced immunogenic activity. Therefore, despite the low price, its use is irrational.
The most effective is the use of purified virus-inactivated concentrates (blood coagulation factor VIII + von Willebrand factor).
Desmopressin
A synthetic analogue of the antidiuretic hormone vasopressin - 1-deamino-8-D-arginine vasopressin (desmopressin) stimulates the release of von Willebrand factor from the depot, leading to an increase in the concentration of the factor in the blood plasma. Desmopressin is most effective in type 1 von Willebrand disease, but an effect is also possible in type 2A. The drug is administered once a day intravenously by drip at a dose of 0.3 mcg / kg in 50-100 ml of isotonic sodium chloride solution for 20-30 minutes, or subcutaneously at the same dose without dilution. There are metered sprays containing highly concentrated desmopressin for intranasal administration at a dose of 150-300 mcg. Long-term use (for several days in a row) leads to the formation of tachyphylaxis due to depletion of von Willebrand factor in the depot. Not recommended for use in children under 3 years of age.
Antifibrinolytics
Aminocaproic acid is administered intravenously by drip at a rate of 100 mg/kg during the first hour, then 30 mg/kg per hour. The maximum daily dose is 18 g. It can be taken orally. Tranexamic acid can be taken orally or intravenously by drip at a dose of 20-25 mg/kg every 8-12 hours. Indications for use: uterine bleeding, bleeding from the mucous membranes of the oral cavity, nasal and gastrointestinal bleeding. Tranexamic acid is used, as a rule, in combination with specific hemostatic treatment, but in mild cases - as the main drug.
In case of urinary tract bleeding, the use of antifibrinolytics is strictly contraindicated due to the risk of obstruction of the urinary tract by blood clots.
Local hemostatic drugs
Local hemostatic drugs - fibrin glue, aminomethylbenzoic acid (hemostatic sponge with amben) and others - are indicated for surgical treatment and in dental practice. Etamsylate (dicynone) is used as an additional hemostatic drug to stop bleeding of various etiologies, often effective for preventing nosebleeds. The drug is administered parenterally at a dose of 3-5 mg / kg 3 times a day. When taken enterally, the dose can be increased by 1.5-2 times.
Complications of von Willebrand disease treatment
The introduction of von Willebrand factor for the purpose of hemostasis in patients with type 3 von Willebrand disease causes the formation of an inhibitor (blocking antibodies) in 10-15% of cases. In case of an inhibitor, the introduction of von Willebrand factor concentrates is contraindicated due to the risk of developing post-infusion anaphylactic reactions.
For hemostasis, it is possible to use recombinant activated concentrate of factor VII (Eptacog alpha activated, NovoSeven) in an average dose of 90 mcg/kg every 2-4 hours until bleeding stops. The use of antifibrinolytics and effects aimed at eliminating the inhibitor (use of hormones, plasmapheresis, intravenous administration of immunoglobulin, etc.) is indicated.
Treatment of acquired von Willebrand disease
Symptomatic treatment and/or prevention of bleeding. In some cases, desmopressin and factor VIII concentrate + von Willebrand factor (blood coagulation factor VIII + von Willebrand factor) are effective. Anti-inhibitor coagulant complex (Feiba Team 4 Immuno) and eptacog [alpha activated] (NovoSeven) may be used. Pathogenetic treatment includes treatment of the underlying disease.
Recommended Doses of Von Willebrand Factor Preparations for Selected Clinical Situations in Children
Nature of bleeding |
Dose, IU/kg |
Number of entries |
Required plasma level |
Major surgeries, adenotonsillotomies (bleeding prevention) |
50-70 |
Once a day |
>50% before reparations |
Minor surgical interventions (bleeding prevention) |
30-60 |
Once a day |
>30-50% before reparations |
Minor surgical interventions (bleeding prevention) |
30-60 |
Once a day |
>30-50% 2-3 days |
Uterine bleeding |
50-80 |
Once a day |
>50% before termination |
Nosebleeds |
30-60 |
One time |
>30-50% |