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Chapter: Medicine and surgery: Haematology and clinical Immunology

von Willebrand disease - Clotting disorders

An inherited coagulation disorder resulting in platelet dysfunction and low levels of circulating factor VIII. - Definition, Incidence, Aetiology, Pathophysiology, Clinical features, Complications, Investigations, Management, Prognosis.

von Willebrand disease

 

Definition

 

An inherited coagulation disorder resulting in platelet dysfunction and low levels of circulating factor VIII.

 

Aetiology/pathophysiology

 

von Willebrand factor (vWF) is a glycoprotein that has two functions in haemostasis. vWF acts as a bridging molecule between allowing platelet aggregation and adhesion to damaged endothelium. vWF acts as a carrier protein for circulating factor VIII increasing the half-life of factor VIII five-fold.

 

Type 1 (autosomal dominant) is the most common type. It causes are reduction in the amount of vWF.

 

Type 2 (usually an autosomal dominant) causes functional abnormalities of vWF. There are at least four subtypes.

 

Type 3 (autosomal recessive) causes extremely reduced or undetectable levels of vWF.

 

Clinical features

 

Type 1 and 2 causes mild disease with bleeding following injury, menorrhagia and epistaxis. Type 3 causes spontaneous bleeding from early life.

 

Investigations

 

Activated partial thromboplastin and prothrombin times are raised, but correctable with 50% normal serum other coagulation measures are normal.

 

Factor VIII:vWF and VIII:c assays are low.

 

Management

 

Treatment of bleeding and prophylaxis in severe disease uses intermediate purity factor VIII concentrates or VIII:vWF concentrates. 1-desamino-8-D-arginine vasopressin (DDAVP) releases factor VIII from endothelial cells and is of value in mild to moderate disease.

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Medicine and surgery: Haematology and clinical Immunology : von Willebrand disease - Clotting disorders |


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