von Willebrand disease in the 21st century: current approaches and new challenges.

摘要

von Willebrand disease (VWD), first described more than 80 years ago, is now recognized as the most common inherited bleeding disorder, with an estimated prevalence of 1.3% in the general population [1]. Clinical manifestations of the disease primarily consist of excessive and prolonged mucocutaneous and postoperative bleeding that result from quantitative or qualitative defects in the adhesive glycoprotein von Willebrand factor (VWF). Accumulating knowledge about the structure and function of this protein, aided by the cloning of the VWF gene in 1985, has paved the way for advances in VWD treatment.