Clinical disorders - Primary hemostasis - Von Willebrand disease

5 important questions on Clinical disorders - Primary hemostasis - Von Willebrand disease

What are the characteristics of VWD type 2? And what are the subtypes of type 2 VWD?

Decreased function of VWF.
Autosomal dominant and 2N is autosomal recessive.
  • 2A: decreased function due to the absence of high molecular weight multimeres
  • 2B: increased affinity for GPIb receptor, reduced number of platelets. VWF binds spontaneously to platelets and when there is a bleeding there is not enough platelets.
  • 2M:  normal size, but VWF does not attach to the platelets normally
  • 2N: VWF does not attach to FVIII normally

What are the treatment options for VWD?

  • DDAVP
  • Tranexamic acid
  • VWF concentrate

What is the working mechanism of DDAVP?

DDAVP stimulates secretion of FVIII and VWF from the Weibel pallade bodies --> plasmaconcentration of VWF will rise. When the Weibel pallade bodies are empty, the medication will not work anymore.
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DDAVP is contra-indicated to which types of VWD?

Type 2B, because the thrombocytopenia will worsen.

It only works for type 1 and some type 2.

What is the working mechanism of tranexaminic acid?

Treatment for mucosal bleedings. It inhibits the fibrinolysis, which means that the clots stay longer. It is usefull in all types of VWD.

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