Textbook of Von Willebrand Disease

List of Contributors vii

Foreword xi

Preface xiii

1 Historical perspective on von Willebrand disease 1
Erik E. Berntorp and Margareta Blombaeck

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 Melanie 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

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• Medicina interna e clínica

VWF structure; VWF function; VWF biosynthesis; VWF organization; VWF modulation by ADAMTS13; VWF clearance; Clinical, laboratory and molecular markers of different VWD types; VWD pediatric; Obstetrics and gynecologic aspects of VWD women; GI bleeds and angiogenesis; plasma-derived and recombinant VWF concentrates; Acquired von Willebrand Syndrome; secondary long-term prophylaxis; risk of thrombosis in VWD