List of Contributors, vii Foreword, xi Preface, xiii 1 Historical perspective on von Willebrand disease, 1 Erik E. Berntorp and Margareta Blombäck 2 Biosynthesis and organization of von Willebrand factor, 7 Sandra L. Haberichter 3 Von Willebrand factor structure and function, 23 Robert R. Montgomery, Veronica H. Flood, and Sandra L. Haberichter 4 Regulation of von Willebrand factor expression, 39 Elham Ghorbanpour and David Lillicrap 5 Modulation of VWF by ADAMTS13, 49 Yaoxian Xu, Anastasis Petri, and James T.B. Crawley 6 Assessment of VWF clearance, 63 Orla Rawley, Laura L.
Swystun, and Jamie M. O'Sullivan 7 Classification of VWD, 73 Jessica Garcia, Sandra L. Haberichter, and Veronica H. Flood 8 The epidemiology of von Willebrand disease, 81 Giancarlo Castaman and Francesco Rodeghiero 9 Clinical aspects of von Willebrand disease: bleeding history, 87 Paula D. James and Alberto Tosetto 10 Laboratory diagnosis of von Willebrand disease: the phenotype, 95 Emmanuel J. Favaloro and Ulrich Budde 11 Molecular diagnosis of von Willebrand disease: the genotype, 109 Pamela Christopherson, Robert R. Montgomery, and Veronica H. Flood 12 Clinical, laboratory, and molecular markers of type 1 von Willebrand disease and low von Willebrand factor, 115 David Lillicrap 13 Clinical and molecular markers of type 1C VWD, 123 Heather Clift, Veronica H.
Flood, and Sandra L. Haberichter 14 Clinical and molecular markers of VWD2A, 129 Reinhard Schneppenheim and Giancarlo Castaman 15 Clinical and molecular markers of VWD2B, 137 Giancarlo Castaman and Augusto B. Federici 16 Clinical and molecular markers of type 2M VWD, 143 Maissaa Janbain, Sandra L. Haberichter, and Veronica H. Flood 17 Clinical and molecular markers of VWD2N, 149 Jenny Goudemand and Mélanie Daniel 18 Clinical, laboratory, and molecular markers of type 3 von Willebrand disease, 159 Luciano Baronciani and Augusto B. Federici 19 Pediatric aspects of von Willebrand disease, 177 Robert F. Sidonio Jr and Jorge Di Paola 20 Women with von Willebrand Disease, 185 Rezan Abdul Kadir 21 The use of desmopressin in von Willebrand disease, 201 Giancarlo Castaman and Augusto B. Federici 22 Plasma-derived and recombinant VWF concentrates, 215 Pier Mannuccio Mannucci and Massimo Franchini 23 Pathophysiology, epidemiology, and management of acquired von Willebrand syndrome, 221 Ulrich Budde and Augusto B.
Federici 24 Cardiovascular causes of AVWS, 239 Antoine Rauch and Sophie Susen 25 Von Willebrand factor regulation of angiogenesis and vascular integrity: implications for gastrointestinal angiodysplasia and beyond, 247 Anna M. Randi and Giancarlo Castaman 26 Prophylaxis in von Willebrand disease, 257 Erik E. Berntorp and Augusto B. Federici 27 Risk of thrombosis and antithrombotic treatment in von Willebrand disease patients, 265 Ferdows Atiq and Frank W.G. Leebeek 28 Novel functions for VWF beyond hemostasis, 271 Ferdows Atiq and James S. O'Donnell Index, 281.