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Textbook of Von Willebrand Disease: Basic and Clinical Aspects

Textbook of Von Willebrand Disease: Basic and Clinical Aspects

9781119419488
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Description

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.

Product Details
Wiley-Blackwell
103040
9781119419488

Data sheet

Publication date
2024
Issue number
2
Cover
hard cover
Pages count
304
Dimensions (mm)
201.00 x 258.00

  • 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

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