Von Willebrand disease (VWD) is a hereditary disease manifested by bleeding disorders. According to statistics, the pathology is diagnosed at a rate of 1 to 2 cases per 10 thousand persons. The cause of the disorder is deficiency or dysfunction of von Willebrand factor, a protein responsible for platelet "clotting". Subject to timely treatment, the vast majority of forecasts on the disease is favorable.
BV can be suspected based on the clinical data and family history. To confirm the diagnosis and exclude similar diseases, the patient is prescribed:
- complete blood count,
- blood chemistry,
- analysis for haematuria,
- abdominal ultrasound,
- integrated CT/MRI,
- bone marrow aspiration biopsy (trepanobiopsy of the ilium wing).
The treatment is carried out with the appearance of bleeding or on the eve of surgical interventions (to prevent complications). To restore the normal blood clotting, the patient is prescribed the transfusion therapy (a single administration of blood products with a high content of von Willebrand factor and coagulation factor VIII). The hemostasis values bounce back 1 to 2 days after the transfusion.
Some manifestations of the disease (bleeding from the nose or from the oral mucosa, uterine bleeding) are docked with additional medicines:
- fibrinolysis inhibitors,
- local hemostatics.
The treatment duration is determined by severity of the condition. Upon cycle completion, the patient is given advice on further treatment. Hide