Von Willebrand Disease: Basic and Clinical Aspects

Von Willebrand Disease: Basic and Clinical Aspects

Hardback

Edited by Augusto B. Federici, Edited by Christine A. Lee, Edited by Erik E. Berntorp, Edited by David Lillicrap, Edited by Robert R. Montgomery

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  • Publisher: Wiley-Blackwell (an imprint of John Wiley & Sons Ltd)
  • Format: Hardback | 268 pages
  • Dimensions: 188mm x 248mm x 20mm | 862g
  • Publication date: 26 April 2011
  • Publication City/Country: Chicester
  • ISBN 10: 1405195126
  • ISBN 13: 9781405195126
  • Edition: 1
  • Sales rank: 1,204,415

Product description

Von Willebrand Disease: Basic and Clinical Aspects provides an insight into all aspects of the condition. Since its discovery, von Willebrand disease has been extensively studied and the causative factor deficiency, the understanding of the condition and its treatment has greatly improved. This book summarizes recent research and will help to optimize the management of patients with von Willebrand disease. This valuable book describes the important and complex role of von Willebrand factor in hemostasis and thrombosis. In addition to the current understanding of its molecular biology, this book gives particular focus to the association between genetic variants of von Willebrand factor and different von Willebrand disease phenotypes. It also reviews the important area of the obstetric and gynecological manifestations of von Willebrand disease, as well as the treatment of acute bleeding. Written by an international team of contributors it describes the progress in, and difficulties of diagnosing phenotypes and genotypes. Molecular diagnosis of type 1, type 2 and its subgroups, and type 3 von Willebrand disease are presented as well as discussion of the potential future role of gene therapy. Von Willebrand Disease: Basic and Clinical Aspects is a valuable resource for hematologists in practice and in training, and specialists in thrombosis and hemostasis.

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Author information

Augusto B. Federici, University of Milan, Milan, Italy Christine A. Lee, Oxford Haemophilia & Thrombosis Centre, Oxford, UK Erik E. Berntorp, Lund University, Malmo, Sweden David Lillicrap, Queen s University, Kingston, ON, Canada Robert R. Montgomery, Medical College of Wisconsin; Milwaukee, WI, USA

Review quote

"This book certainly helped to enhance my knowledge about von Willebrand disease and even prompted me to present a review of the disease to my colleagues. It is excellently written, appeals to a broad category of readers, and exceeds its stated objectives. I would definitely recommend the book to colleagues." (Doody's, 23 September 2011)

Back cover copy

Von Willebrand Disease: Basic and Clinical Aspects provides an insight into all aspects of the condition. Since its discovery, von Willebrand disease has been extensively studied and the causative factor deficiency, the understanding of the condition and its treatment has greatly improved. This book summarizes recent research and will help to optimize the management of patients with von Willebrand disease. This valuable book describes the important and complex role of von Willebrand factor in hemostasis and thrombosis. In addition to the current understanding of its molecular biology, this book gives particular focus to the association between genetic variants of von Willebrand factor and different von Willebrand disease phenotypes. It also reviews the important area of the obstetric and gynecological manifestations of von Willebrand disease, as well as the treatment of acute bleeding. Written by an international team of contributors it describes the progress in, and difficulties of diagnosing phenotypes and genotypes. Molecular diagnosis of type 1, type 2 and its subgroups, and type 3 von Willebrand disease are presented as well as discussion of the potential future role of gene therapy. Von Willebrand Disease: Basic and Clinical Aspects is a valuable resource for hematologists in practice and in training, and specialists in thrombosis and hemostasis.

Table of contents

Contributors vii Foreword xi Preface xiii 1 Historical perspective on von Willebrand disease 1 Erik Berntorp and Margareta Blomback 2 Biosynthesis and organization of von Willebrand factor 7 Sandra L. Haberichter 3 von Willebrand factor structure and function 30 Robert R. Montgomery and Sandra L. Haberichter 4 Modulation of von Willebrand factor by ADAMTS13 49 Jennifer Barr and David Motto 5 Animal models in von Willebrand disease 63 Cecile V. Denis, Olivier D. Christophe and Peter J. Lenting 6 Classifi cation of von Willebrand disease 74 Javier Batlle, Almudena Perez-Rodriguez and Maria Fernanda Lopez-Fernandez 7 The epidemiology of von Willebrand disease 86 Giancarlo Castaman and Francesco Rodeghiero 8 Clinical aspects of von Willebrand disease: bleeding history 91 Paula D. James and Alberto Tosetto 9 Laboratory diagnosis of von Willebrand disease: the phenotype 100 Ulrich Budde and Emmanuel J. Favaloro 10 Molecular diagnosis of von Willebrand disease: the genotype 114 Anne C. Goodeve and Reinhard Schneppenheim 11 Clinical, laboratory, and molecular markers of type 1 von Willebrand disease 127 David Lillicrap, Francesco Rodeghiero, and Ian Peake 12 Clinical, laboratory, and molecular markers of type 2 von Willebrand disease 137 Dominique Meyer, Edith Fressinaud, and Claudine Mazurier 13 Clinical, laboratory, and molecular markers of type 3 von Willebrand disease 148 Luciano Baronciani, Augusto B. Federici, and Jeroen C.J. Eikenboom 14 Pediatric aspects of von Willebrand disease 166 Jorge Di Paola and Thomas Abshire 15 Women with von Willebrand disease 174 Christine A. Lee, Rezan A. Kadir, and Peter A. Kouides 16 On the use of desmopressin in von Willebrand disease 186 Stefan Lethagen, Augusto B. Federici, and Giancarlo Castaman 17 The use of plasma-derived concentrates 200 Pier Mannuccio Mannucci and Massimo Franchini 18 Prophylaxis in von Willebrand disease 207 Erik Berntorp 19 Pathophysiology, epidemiology, diagnosis, and treatment of acquired von Willebrand syndrome 214 Ulrich Budde, Augusto B. Federici and Jacob H. Rand 20 Gene therapy for von Willebrand disease 231 Marinee K.L. Chuah, Inge Petrus, and Thierry Vanden Driessche Index 245