von Willebrand's Disease

Von Willebrand's disease is the commonest inherited bleeding disorder. One percent of the population has a decrease in vWF but less than 10% of these individuals have a significant bleeding disorder. Functional vWF is present in normal plasma as multimers that bind to platelets and to collagen forming a bridge which forms the basis for early hemostasis. If the multimers are too few or too small they cannot subserve this hemostatic function. If, on the other hand, the multimers are too large and too reactive they can be instumental in causing the devastating disease TTP. This chapter sets out to review our present understanding of vWF and its role in disease.

Author(s): Peter R. Galbraith MD, FRCP(C)




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