alexa Von Willebrand Disease | Belgium| PDF | PPT| Case Reports | Symptoms | Treatment

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Von Willebrand Disease

  • Von Willebrand Disease

    Von Willebrand disease (vWD) is an inherited trait where an individual’s bleeds excessively. It is very rare that the vWD is acquired later in life due to autoantibodies. The impairment of protein called von Willebrand factor which is an important component in blood–clotting process. The vWF gene is located on chromosome 12. Types 1 and 2 are inherited as autosomal dominant traits and type 3 is inherited as autosomal recessive. Occasionally type 2 also inherits recessively.

  • Von Willebrand Disease

    Disease statistics: VWD has a prevalence of about 1% in the general population, but the figure for clinically relevant cases is lower (about 100/million inhabitants). Bleeding manifestations are heterogeneous: mucosal bleeding is typical of all VWD cases but hemarthrosis and hematomas may also be present when FVIII levels are low. Partial quantitative deficiency of VWF (type 1 VWD); qualitative variants (type 2 VWD), due to a dysfunctional VWF, are clinically more homogeneous.

  • Von Willebrand Disease

    Treatment: The two main treatment possibilities for patients with von Willebrand disease (vWD) are desmopressin (DDAVP) and von Willebrand factor/factor VIII (vWF/FVIII) concentrates. DDAVP is a synthetic analogue of the antidiuretic hormone vasopressin; it has enhanced antidiuretic activity and no pressor activity related to vasopressin. Purified plasma-derived concentrates of vWF/FVIII are used for treatment of bleeds and for surgical prophylaxis when DDAVP is ineffective or contraindicated.

  • Von Willebrand Disease

    Research: This research carried in by the Hemostasis Unit of the Antwerp University Hospital (UZA) and support by Belgian Society on Thrombosis & Hemostasis (BSTH) during the last few years, have been made to optimize existing therapies for VWD, but also to devise new approaches, such as inducing endogenous expression with interleukin-11, administering exogenous recombinant VWF, or introducing the protein via gene delivery.

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