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What
is von Willebrand disease?
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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 Willebrand’s
identified in the UK, but more may be as yet undiagnosed,
since the von Willebrand’s disorder is believed to
affect 1% of the population.
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Are
there any other factors?
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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.
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Factor II (prothrombin) deficiency:
Nosebleeds, heavy periods, and easy bruising are
features. Joint bleeds are rare.
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Factor V deficiency:
Easy bruising, nosebleeds, heavy periods, and joint
and muscle bleeds are experienced by severely affected
people.
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Factor VII deficiency:
Effects are usually mild but include nosebleeds,
heavy periods, and bruising. Joint bleeds are rare.
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Factor X deficiency:
Bruising, joint bleeds, and heavy periods occur
in people with this deficiency.
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Factor XI deficiency:
Affected people often have Jewish ancestry. Nosebleeds
and heavy periods are experienced.
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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.
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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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