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Other Bleeding Disorders

What is von Willebrand disease?

von Willebrand disease is an inherited bleeding disorder, similar to Haemophilia, but the two disorders are not the same. vWD is the most common inherited clotting disorder, affecting both men and women. It has been estimated that vWD affects up to one percent of the population.  However, it is generally the least severe of the clotting disorders.

vWD is caused by a deficiency or defect of a blood clotting protein called von Willebrand factor (vWF), a gluelike protein that helps platelets in the blood stick together and seal off tears in injured blood vessels. This is called a platelet plug. If a person does not have enough vWF or it does not work properly, no platelet plug will form and bleeding will continue for a longer period of time.

Most people with vWD will have few, if any, symptoms. The main symptoms are easy bruising, frequent or prolonged nosebleeds, heavy or prolonged menstrual bleeding, and prolonged bleeding following injury, surgery, dental work, or childbirth.

vWD is treated with desmopressin (also called DDAVP) or with infusions of a clotting factor concentrate that contains vWF.

There are about 5,000 people with von Willebrandís identified in the UK, but more may be as yet undiagnosed, since the von Willebrandís disorder is believed to affect 1% of the population.

Are there any other factors?

With Haemophilia A there is a factor VIII deficiency, with Haemophilia B there is a factor IX deficiency and with von Willebrand's disease there is a von Willebrand's factor (and sometimes factor VIII) deficiency.

There are also a number of related bleeding disorders where there are other factor deficiencies that are much rarer than haemophilia. The effects will depend, like haemophilia, on the degree of the missing factor. Without treatment, prolonged bleeding may follow surgery, childbirth, or major injury.

  • Factor I (fibrinogen) deficiency:

    Joint bleeds are uncommon, but there is more likelihood of oozing from small wounds than in haemophilia. Easy bruising will occur.

  • Factor II (prothrombin) deficiency:

    Nosebleeds, heavy periods, and easy bruising are features. Joint bleeds are rare.

  • Factor V deficiency:

    Easy bruising, nosebleeds, heavy periods, and joint and muscle bleeds are experienced by severely affected people.

  • Factor VII deficiency:

    Effects are usually mild but include nosebleeds, heavy periods, and bruising. Joint bleeds are rare.

  • Factor X deficiency:

    Bruising, joint bleeds, and heavy periods occur in people with this deficiency.

  • Factor XI deficiency:

    Affected people often have Jewish ancestry. Nosebleeds and heavy periods are experienced.

  • Factor XII deficiency:

    This deficiency is usually diagnosed only when blood taken for some other reason fails to clot in the normal time. Surprisingly, people with even a severe deficiency have little or no trouble from bleeding.

  • Factor XIII deficiency:

    Clots form in the normal time but tend to break down later as they are not stabilised. This leads to a delay in healing after injury or surgery. Females with severe XIII deficiency have frequent miscarriages. Factor XIII deficiency may present early in life with oozing from the umbilical cord. People with severe factor XIII deficiency should be treated with monthly prophylaxis with factor XIII concentrate. This prevents bleeding altogether.


Breathofair would like to thank
World Federation of Haemophilia
and Haemophilia Society
for the information on this site regarding Haemophilia

 
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