Textbook of Von Willebrand Disease

Comprehensive resource summarizing recent research on von Willebrand disease, showing clinicians how to optimize management of patients with this disorder The newly revised and updated second edition of Textbook of von Willebrand Disease: Basic and Clinical Aspects, 2nd edition describes the important and complex role of von Willebrand factor (VWF) in hemostasis and thrombosis, covering not only the current understanding of its molecular biology, but also the association between genetic variants of VWF and different von Willebrand disease (VWD) phenotypes. The text also reviews the important area of the obstetric and gynecological manifestations of VWD, as well as how to manage patients with VWD for surgery. Many advances in agents are included in this updated edition, as well as the wide topics such as VWF in Angiogenesis, and VWF/ADAMTS13 as risk factors of thrombosis. Edited by a team of experts in VWD and an international team of contributors, Textbook of von Willebrand Disease covers sample topics such as: VWF structure and function, biosynthesis and organization of VWF, modulation of VWF by ADAMTS13 and assessment of VWF clearance Clinical, laboratory and molecular markers of different VWD types, from the mild forms of type 1 VWD and the moderate variants types 2A, 2B, 2M, 2N to the most severe type 3 VWD Pediatric aspects of VWD and of women with VWD Management of GI bleeds and appropriate therapies in surgery, and plasma-derived and recombinant VWF concentrates Acquired von Willebrand Syndrome, cardiovascular causes of AVWS, gastrointestinal bleeds in VWD and AVWS, and prophylaxis in von Willebrand disease Providing complete and accessible coverage of the subject, Textbook of von Willebrand Disease: Basic and Clinical Aspects, 2nd edition is a valuable resource for hematologists in practice and in training, along with specialists in thrombosis, hemostasis, and bleeding/clotting disorders.

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 vonWillebrand 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 forgastrointestinal 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