Current Knowledge on Von Willebrand's Disease

Abstract

Von Willebrand's disease (vWD) is an inherited bleeding disorder due to deficiency or abnormality of von Willebrand factor (vWF), a large multimeric glycoprotein which forms a noncovalent complex with factor VIII in blood. On the basis of the structure-function relationship of F. VIII/vWF complex, the disease can be divided into three main states; type I (classical form), type II (variant form) and type III (severe, homozygous form). A bleeding disorder with an intrinsic platelet abnormality affecting platelet-vWF interaction is called pseudo-vWD or platelet type vWD.
This paper will review 1) Structure function relationship of vWF; 2) Diagnosis of vWD; 3) Ristocetin induced platelet aggregation and Botrocetin induced platelet aggregation in various types of vWD; 4) Plasma multimeric composition of vWF in various types of vWD; 5) vWF-subunit structure in various types of vWD; and 6) Hemostatic effects of DDAVP and heated factor VIII concentrates.