Introduction

When Eric von Willebrand, almost 80 years ago, first described a family with a new hemorrhagic disease, he could hardly imagine that his seminal discover was opening new frontiers of research in a fascinating area of investigation, still very active and fruitful at the beginning of the third millennium. Moving from the recognition, in early ’70, that the intriguing bleeding disorder called after his name (von Willebrand disease, VWD) is caused by quantitative or qualitative defects of the plasma protein von Willebrand factor (VWF), we are now understanding more and more its molecular structure, functional domains, multimeric assembly and pleiotropic functions.