A Rare Case of Type III Von Willebrand Disease in a Woman of Reproductive Age

Abstract

Von Willebrand disease (vWD) is the most comman inherited bleeding disorderr. Von Willebrand's factor (vWF) is a glycoprotein produced by' vascular endothelium with a molecular weight about 200000. It froms multimers in the circrtlation2. Von Willebrand's factor has two main functions. It stabilizes factor VIII in circulation and promotes adhesion of platelets to damaged vasctdar endothelium. The' gene for vWF is located in chromosome 12 and genetic mutation of this gene calrses vWD. There are 3 different types of vWD Type I and II has autosomal dominant inheri- tance pattern and type III the most severe form has an antosomal recessive inheritance3. Type I is the most common mildest form present in approdmately 75o/" of patients. It is associated with a quantitative deficiency of vWF. In type II the defect is qualitative. In these cases often a family history of mucocutaneous bleeding and perscnal history of mucocutaneous bleeding tendency and menorrhagia often occur.,Major haemorrhages are rare. Type III disease is associated with a negligible amount of vWF {both qrlantity and fttnction affected) and therefore a significant reduction in factorVlll activity. In these cases tire bleeding history is severe and can cause life threatening haemorrhagesa.