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.
Table of Contents
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
