Von Willebrand disease

From WikiLectures


Von Willebrand's disease is one of the most common congenital blood clotting disorders. It affects 1 in 1,000 people (women as often as men). Mostly, however, these are mild diseases without more serious clinical manifestations. It was first described by the Finnish physician Erik von Willebrand in 1924, but it was not until the 1950s that deficiency or malfunction of von Willebrand factor (vWF) was shown to be the cause.

Causes[edit | edit source]

  • autosomal dominant inheritance mutation of the gene encoding the amount, functionality and structure of vWF impaired platelet function, inability to bind plasma factor VIII
  • the obtained forms were also described

Symptoms[edit | edit source]

  • vWF is not among the plasma factors it is not a coagulopathy
  • impaired blood clotting – frequent nosebleeds (epistaxis), increased formation of bruises, heavier menstrual bleeding, there may be blood in the urine and stools, in severe forms bleeding into the joints

Basic examination[edit | edit source]

  • blood count
  • APTT
  • Duke bleeding test
  • examination of factor VIII
  • tests performed may not be pathological in milder forms

Duke:


Classification[edit | edit source]

Type 1
light quantitative defect – the most common
Type 2
qualitative defect
Type 3
severe qualitative defect – the most severe form

Links[edit | edit source]

Related Articles[edit | edit source]

Resources[edit | edit source]