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Treatment of von Willebrand disease
Last reviewed: 20.11.2021
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Like any hereditary disease, you can not cure Willebrand's disease; Only treatment or prevention of manifestations of the disease is possible.
Specific haemostatic effects (von Willebrand factor concentrates, desmopressin).
VWF preparations are indicated for type 3 and severe course of other types of Willebrand disease.
Introduction FFP in von Willebrand disease with haemostatic purpose is not rational because of the relatively low concentration of von Willebrand factor in it. The blood clotting factor VIII (cryoprecipitate) contains 10 times the von Willebrand factor per unit volume. Its shortcomings 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 use of purified virus-inactivated concentrates (factor of blood coagulation VIII + von Willebrand factor).
Desmopressin
Synthetic analogue of the antidiuretic hormone vasopressin - 1-deamino-8-B-arginine vasopressin (desmopressin) stimulates the release of von Willebrand factor from the depot, which leads to an increase in the concentration of the factor in the blood plasma. The most effective use of desmopressin in type 1 von Willebrand disease, but the effect is also possible with type 2A. The drug is administered once a day intravenously drip at a dose of 0.3 μg / kg in 50-100 ml of isotonic sodium chloride solution for 20-30 minutes, or subcutaneously in the same dose without dilution. There are metered sprays containing highly concentrated desmopressin for intranasal administration at a dose of 150-300 μg. Prolonged use (for several days in a row) leads to the formation of tachyphylaxis due to depletion of von Willebrand factor in the depot. Do not recommend use in children under 3 years.
Antifibrinolytics
Aminocaproic acid is administered intravenously drip from the calculation of 100 mg / kg for the first hour, then 30 mg / kg per hour. The maximum daily dose is 18 g. You can take it inside. Tranexamic acid can be taken orally or intravenously drip in a dose of 20-25 mg / kg every 8-12 hours Indications for use: uterine bleeding, bleeding from the mucous membranes of the mouth, nasal and gastrointestinal bleeding. Tranexamic acid is used, as a rule, in combination with specific hemostatic treatment, but in mild cases - the main drug.
With bleeding from the urinary tract, the use of antifibrinolytic drugs is categorically contraindicated because of the risk of obturation with blood clots of the urinary tract.
Local haemostatic preparations
Local haemostatic preparations, - fibrin glue, aminomethylbenzoic acid (hemostatic sponge with ambene) and others, are shown in operative treatment and in dental practice. Etamsylate (dicinone) is used as an additional haemostatic drug for the relief of bleeding of various etiologies, often effective for the prevention of nasal bleeding. The drug is administered parenterally at a dose of 3-5 mg / kg 3 times a day. With enteral administration, the dose can be increased by a factor of 1.5-2.
Complications of treatment for von Willebrand disease
The introduction of von Willebrand factor for the purpose of hemostasis in patients with type 3 Willebrand disease in 10-15% of cases causes the formation of an inhibitor (blocking antibodies). With an inhibitor, the introduction of von Willebrand factor concentrates is contraindicated because of the risk of postinfusion anaphylactic reactions.
For hemostasis, it is possible to use recombinant activated factor VII concentrate (eptactog alpha activated, NovoSeven) at an average dose of 90 mcg / kg every 2-4 h until bleeding stops. The use of antifibrinolytic agents and effects aimed at eliminating the inhibitor (use of hormones, plasmapheresis, intravenous immunoglobulin, etc.) is shown.
Treatment of acquired von Willebrand disease
Symptomatic effects and / or prevention of bleeding. In some cases, the use of desmopressin and concentrate factor VIII + von Willebrand factor (coagulation factor VIII + von Willebrand factor) is effective. It is possible to use anti-inhibitory coagulant complex (Feiba Tim 4 Immuno) and eptacog [alpha activated] (NovoSeven). Pathogenetic treatment involves affecting the underlying disease.
Recommended doses of von Willebrand factor preparations for some clinical situations in children
|
Bleeding pattern |
Dose, IU / kg |
Number of Inputs |
Required level in blood plasma |
|
Large surgery, adenotonzillotomii (prevention of bleeding) |
50-70 |
Once a day |
> 50% before the onset of repair |
|
Small surgical interventions (bleeding prophylaxis) |
30-60 |
Once a day |
> 30-50% before the onset of repair |
|
Small surgical interventions (bleeding prophylaxis) |
30-60 |
Once a day |
> 30-50% 2-3 days |
|
Uterine bleeding |
50-80 |
Once a day |
> 50% before termination |
|
Nasal bleeding |
30-60 |
Once |
> 30-50% |