Von Willebrand Disease Basic and Clinical Aspects

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

• ISBN13: 9781405195126
• ISBN10: 1405195126
• Edition: 1st
• Format: Hardcover
• Copyright: 2011-04-18
• Publisher: Wiley-Blackwell

Summary

This book provides an insight into all aspects of von Willebrand disease. It describes the important and complex role of von Willebrand factor in haemostasis and thrombosis, in addition to our current understanding of its molecular biology, with a particular focus on the association between genetic variants of von Willebrand factor and these disease phenotypes. It also reviews the important area of the obstetric and gynaecological manifestations of von Willebrand disease, as well as the treatment of urgent bleeding.

Author Biography

Augusto B. Federici, University of Milan, Milan, Italy

Christine A. Lee, Oxford Haemophilia & Thrombosis Centre, Oxford, UK

Erik E. Berntorp, Lund University, Malmö, Sweden

David Lillicrap, Queen’s University, Kingston, ON, Canada

Robert R. Montgomery, Medical College of Wisconsin; Milwaukee, WI, USA

Table of Contents

Contributors.

Foreword.

Preface.

1 Historical perspective on von Willebrand disease (Erik Berntorp and Margareta Blombäck).

2 Biosynthesis and organization of von Willebrand factor (Sandra L. Haberichter).

3 von Willebrand factor structure and function (Robert R. Montgomery and Sandra L. Haberichter).

4 Modulation of von Willebrand factor by ADAMTS13 (Jennifer Barr and David Motto).

5 Animal models in von Willebrand disease (Cécile V. Denis, Olivier D. Christophe and Peter J. Lenting0.

6 Classifi cation of von Willebrand disease (Javier Batlle, Almudena Pérez-Rodríguez and María Fernanda López-Fernández).

7 The epidemiology of von Willebrand disease (Giancarlo Castaman and Francesco Rodeghiero).

8 Clinical aspects of von Willebrand disease: bleeding history (Paula D. James and Alberto Tosetto).

9 Laboratory diagnosis of von Willebrand disease: the phenotype (Ulrich Budde and Emmanuel J. Favaloro).

10 Molecular diagnosis of von Willebrand disease: the genotype (Anne C. Goodeve and Reinhard Schneppenheim).

11 Clinical, laboratory, and molecular markers of type 1 von Willebrand disease (David Lillicrap, Francesco Rodeghiero, and Ian Peake).

12 Clinical, laboratory, and molecular markers of type 2 von Willebrand disease (Dominique Meyer, Edith Fressinaud, and Claudine Mazurier).

13 Clinical, laboratory, and molecular markers of type 3 von Willebrand disease (Luciano Baronciani, Augusto B. Federici, and Jeroen C.J. Eikenboom).

14 Pediatric aspects of von Willebrand disease (Jorge Di Paola and Thomas Abshire).

15 Women with von Willebrand disease (Christine A. Lee, Rezan A. Kadir, and Peter A. Kouides).

16 On the use of desmopressin in von Willebrand disease (Stefan Lethagen, Augusto B. Federici, and Giancarlo Castaman).

17 The use of plasma-derived concentrates (Pier Mannuccio Mannucci and Massimo Franchini).

18 Prophylaxis in von Willebrand disease (Erik Berntorp).

19 Pathophysiology, epidemiology, diagnosis, and treatment of acquired von Willebrand syndrome (Ulrich Budde, Augusto B. Federici and Jacob H. Rand).

20 Gene therapy for von Willebrand disease (Marinee K.L. Chuah, Inge Petrus, and Thierry VandenDriessche).

Index.