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Von willlebrand’s disease: Presentation,Diagnosis & Treatment

(HIGH YIELD NOTES FOR |MBBS|NEETPG|NEXT|INICET|FMGE)

 

An autosomal dominant condition in which patients have deficient or defective von Willebrand’s factor (vWF) with low levels of factor VIII, which is carried by vWF.

Symptoms are due to platelet dysfunction and to deficient factor VIII.

The disease is milder than hemophilia.

vWD is the most common inherited bleeding disorder

 

 

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Presentation:

Presents with easy bruising, mucosal bleeding (eg, epistaxis, oral bleeding), menorrhagia, and postincisional bleeding.

Platelet dysfunction is not severe enough to produce petechiae.

Ristocetin cofactor assay measures the ability of vWF to agglutinate platelets in vitro in the presence of ristocetin.

 

 

Treatment:

Bleeding episodes can be treated with DDAVP;

menorrhagia can be controlled with OCPs.

Avoid ASA and other inhibitors of platelet function.

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(ASA ↑ the risk of bleeding in patients with vWD)

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