Von Willebrand Disease - Therapy

Therapy

For patients with vWD type 1 and vWD type 2A, Desmopressin (DDAVP) is recommended for use in case of minor trauma or in preparation for dental or minor surgical procedures. Desmopressin (DDAVP) stimulates release of von Willebrand factor from the Weibel Palade bodies of endothelial cells and increases the levels of vWF as well as coagulant Factor VIII 3-5 fold. Desmopressin (DDAVP) is available as preparation for intranasal administration (Stimate) as well as preparation for intravenous administration.

Desmopressin (DDAVP) is contraindicated in vWD type 2b because of the risk of aggravated thrombocytopenia as well as thrombotic complication.

Desmopressin (DDAVP) is probably not effective in vWD type 2M and is rarely effective in vWD type 2N. It is totally ineffective in vWD type 3.

For women with heavy menstrual bleeding, estrogen-containing oral contraceptive medications are effective in reducing the frequency and duration of the menstrual periods. Estrogen compounds available for use in the correction of menorrhagia are Ethinyl Estradiol and Levonorgestel (levona, Nordette, Lutera, Trivora). Administration of Ethinyl Estradiol diminishes the secretion of luteinizing hormone and follicle stimulating hormone from the pituitary, leading to stabilization of the endometrial surface of the uterus.

Desmopressin (DDAVP) is a synthetic analog of the natural antidiuretic hormone Vasopressin. Overuse of Desmopressin (DDAVP) can lead to water retention and dilutional hyponatremia with consequent convulsion.

For patients with vWD scheduled for surgery and cases of vWD disease complicated by clinically significant hemorrhage, human derived medium purity Factor VIII concentrates, which also contain von Willebrand factors, are available for prophylaxis and treatment. Humate P, Alphanate and Koate HP are commercially available for prophylaxis and treatment of von Willebrand disease. Monoclonally purified Factor VIII concentrates and recombinant Factor VIII concentrates contain insignificant quantity of VWF and are therefore not clinically useful.

Development of alloantibodies occur in 10-15% of patients receiving human derived medium purity Factor VIII concentrates and the risk of allergic reactions including anaphylaxis must be considered when administering these preparations. Administration of the latters is also associated with increased risk of venous thromboembolic complications.

Blood transfusions are given as needed to correct anemia and hypotension secondary to hypovolemia.

Infusion of platelet concentrates is recommended for correction of hemorrhage associated with platelet-type von Willebrand disease.

The antifibrinolytic agents Epsilon amino caproic acid and Tranexamic acid are useful adjuncts in the management of vWD complicated by clinical hemorrhage.

The use Topical thrombin JMI and Topical Tisseel VH are effective adjuncts for correction of hemorrhage from wounds.