Von-Willebrand Disease Flashcards Preview

Haematology > Von-Willebrand Disease > Flashcards

Flashcards in Von-Willebrand Disease Deck (15)
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1
Q

What is von Willebrands Disease?

A

In VWD, there is defective platelet function as well as factor VIII:C deficiency, and both are due to a deficiency or abnormality of VWF. VWF plays a role in platelet adhesion to damaged subendothelium as well as stabilizing factor VIII in plasma

2
Q

What gene is VWF located on?

A

Chromosome 12

3
Q

What is type 1 VWD?

A

A partial quantitative deficiency of VWF - significant type 1 VWD is usually inherited as an autosomal dominant disorder

4
Q

What is type 2 VWD?

A

Due to a qualitative abnormality of VWF, and it too is usually inherited as an autosomal dominant.

5
Q

What is type 3 VWD?

A

Recessively inherited and patients have virtually complete deficiency of VWF.

Their parents are often phenotypically normal

6
Q

How do those with VWD present?

A

Type 1 and type 2 - relatively mild features

  • Bleeding follows minor trauma or surgery
  • Epistaxis
  • Menorrhagia often occur

Type 3 - more severe bleeding

7
Q

What are signs of VWD?

A

Platelet disorder

  • Brusing
  • Epistaxis
  • Menorrhagia
8
Q

What investigations would you do if you suspected VWD?

A
  • FBC - platelets
  • LFTs
  • Clotting Screen - Factors, Bleeding time, PT, APTT, Thrombin time, INR
9
Q

What might you find when you perform APTT in someone with VWD?

A

Increased APTT - due to factor VIII deficiency

10
Q

What might you find when you perform PT on someone with VWD?

A

Normal PT

11
Q

What might you find when you perform FBC on someone with VWD?

A
  • Normal platelet count
  • Normal Hb
12
Q

What might you find on performing a bleeding time assay in someone with VWD?

A

Increased bleeding time

13
Q

What might you find if you performed an assay which looked at someones clotting factors in someone with VWD?

A
  • Decreased VWF
  • Decreased VIIIc
14
Q

How would you manage someone with vWD?

A
  • DDAVP - mild bleeding
  • vWF concentrate containing factor VIII
  • Tranexamic Acid
  • Topical applications
  • OCP
15
Q

What should you avoid the use of in vWD?

A

NSAID use