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Von Willebrand Disease (VD)

Von Willebrand Disease (VD) is the most common hereditary bleeding disorder. Most cases are mild. Bleeding may occur after surgery or when you have a tooth pulled. Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) can make this condition worse. Bleeding may decrease during pregnancy. Von Willebrand factor helps platelets to clump together and stick to the blood vessel wall, which is necessary for normal blood clotting.

Von Willebrand's is mostly a genetic condition. This means it is passed down through the genes from parent to child. The abnormal gene in is on one of the regular chromosomes, not on one of the chromosomes (like haemophilia).Many people carrying this gene are asymptomatic which means that although they will not have bleeding symptoms, they can still pass the condition on to their children, who could have more severe symptoms than their parents. High molecular weight multimers have higher numbers of platelet-binding sites and greater adhesive properties. Each multimeric subunit has binding sites for the receptor glycoprotein Ib on nonactivated platelets and the receptor glycoprotein IIb/IIIa on activated platelets.

This facilitates both platelet adhesion and platelet aggregation, making high molecular weight multimers most important for normal platelet function. Von Willebrand Disease is divided into three categories. Type 1 is characterized by a partial quantitative decrease of qualitatively normal VWF and FVIII. Type 2 VWD the amount of VWF in people's blood is often normal. The problem is that the VWF does not work properly.

There are several sub-types of Type 2 VWD. It is important to get an exact diagnosis because the sub-types can be treated differently. Analysis of VWF multimers reveals a relative reduction in intermediate and high molecular weight multimer complexes. Type 3 is characterized by severe clinical bleeding and is inherited as an autosomal recessive trait. Consanguinity is common in kindreds with this variant. Less severe clinical abnormalities and laboratory abnormalities may be identified in occasional heterozygotes; however, such cases are difficult to identify. Multimeric analysis of the small amount of VWF present yields variable results, in some cases revealing only small multimers.

Causes of Von Willebrands Disease

Common causes of Von Willebrands Disease

  • Genetic disorder.
  • Wilms tumor.
  • Congenital heart disease.
  • Systemic lupus erythematosus.
  • Angiodysplasia.
  • Seizure disorders.
  • Hypothyroidism.

Symptoms of Von Willebrands Disease

Common Symptoms of Von Willebrands Disease

  • Bleeding tendency.
  • Joint bleeding .
  • Bruising.
  • Nosebleeds.
  • Skin rash.
  • Heavy menstrual periods(in women)
  • Blood loss during childbirth.

Treatment of Von Willebrands Disease

Common Treatment of Von Willebrands Disease

  • Partial quantitative decrease of qualitatively normal VWF and FVIII usually are treated with desmopressin.
  • Tranexamic acid and similar drugs : these drugs help to clots being broken down once they've formed.
  • Thrombin : a natural clotting agent.
  • Concentrate of VWF and Factor VIII. These compounds, derived from blood, are used in more serious Von Willebrands
  • Oral contraceptives (or birth control pills), which can help women who have heavy menstrual bleeding.
  • Antifibrinolytic drugs( Alphanate, Humate-P and Amicar )which help prevent the breakdown of clots that form at bleeding sites. These drugs are used mostly to stop or prevent bleeding.





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