Von WIllebrand's Disease Flashcards

1
Q

What is vWD.

A

It is the most common inherited disorder of coagulation.

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2
Q

What is the predominant aetiology of vWD.

A

There are various subtypes of the disease, but the most common is an autosomal dominant conditions.

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3
Q

What is vWD associated with. (2)

A

Either low or abnormal vWF.

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4
Q

What are the symptoms of vWD. (5)

A
Mucosal/capillary bleeding (eg increased bleeding post-tooth extraction). 
Easy bruising. 
Epistaxis. 
Menorrhagia. 
GI haemorrhage.
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5
Q

What is found on the blood results of a patient with vWD. (5)

A
Raised APTT. 
No change in PT. 
Decreased Factor VIIIc and vWF. 
Increased bleeding time. 
Increased bleeding time.
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6
Q

What are the subtypes of vWD. (3)

A

Type 1 and type 3 are quantitative.

Type 2 is qualitative.

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7
Q

What does vWF act as a carrier protein for.

A

Factor VIII.

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8
Q

What is the pathophysiology of vWD. (2)

A

Impaired platelet plug formation.

Abnormally low levels of factor VIII.

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9
Q

Which blood group has a naturally low level of vWF.

A

Group O.

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10
Q

What is the most common aetiology of vWD.

A

Type 1 quantitative disorder.

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11
Q

What are the main roles of vWF. (2)

A

Plays a role in platelet adhesion to damaged subendothelium.
A stabalizing factor for factor VIII in plasma.

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12
Q

When does bleeding usually occur in patients with vWD.

A

Usually following surgery or trauma.

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13
Q

What symptom from haemophilia A are not present in vWD.

A

Haemarthroses are rare in vWD.

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14
Q

What are the normal roles of vWF. (3)

A

To bring platelets into contact with exposed subendoethelium.
To make platelets bind to each other.
To bind to Factor VIII, protecting it from destruction in the circulation.

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15
Q

How many types of vWD are there.

A

More than 22.

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16
Q

What percentage of vWD are type 1.

A

60-80%.

17
Q

What percentage of vWD are type 2.

A

20-30%.

18
Q

What percentage of vWD are type 3.

A

1-5%.

19
Q

Which subset of vWD causes the most severe symptoms.

A

Type 3.