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
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
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).
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