Professor Augusto B. Federici, Hematology and Transfusion Medicine, L. Sacco University Hospital and University of Milan, Milan, Italy. Professor Erik E. Berntorp, Department of Translational Medicine, Malmö Centre for Thrombosis and Haemostasis, Lund University, Skåne University Hospital, Malmö, Sweden. Professor David Lillicrap, Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen’s University, Kingston, ON, Canada. Professor Robert R. Montgomery, Pediatrics (Hematology) and Vice Chair, Versiti Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI, USA.

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• List of Contributors viiForeword xiPreface xiii1 Historical perspective on von Willebrand disease 1Erik E. Berntorp and Margareta Blombäck2 Biosynthesis and organization of von Willebrand factor 7Sandra L. Haberichter3 Von Willebrand factor structure and function 23Robert R. Montgomery Veronica H. Flood and Sandra L. Haberichter4 Regulation of von Willebrand factor expression 39Elham Ghorbanpour and David Lillicrap5 Modulation of VWF by ADAMTS13 49Yaoxian Xu Anastasis Petri and James T.B. Crawley6 Assessment of VWF clearance 63Orla Rawley Laura L. Swystun and Jamie M. O’Sullivan7 Classification of VWD 73Jessica Garcia Sandra L. Haberichter and Veronica H. Flood8 The epidemiology of von Willebrand disease 81Giancarlo Castaman and Francesco Rodeghiero9 Clinical aspects of von Willebrand disease: bleeding history 87Paula D. James and Alberto Tosetto10 Laboratory diagnosis of von Willebrand disease: the phenotype 95Emmanuel J. Favaloro and Ulrich Budde11 Molecular diagnosis of von Willebrand disease: the genotype 109Pamela Christopherson Robert R. Montgomery and Veronica H. Flood12 Clinical laboratory and molecular markers of type 1 von Willebrand disease and low vonWillebrand factor 115David Lillicrap13 Clinical and molecular markers of type 1C VWD 123Heather Clift Veronica H. Flood and Sandra L. Haberichter14 Clinical and molecular markers of VWD2A 129Reinhard Schneppenheim and Giancarlo Castaman15 Clinical and molecular markers of VWD2B 137Giancarlo Castaman and Augusto B. Federici16 Clinical and molecular markers of type 2M VWD 143Maissaa Janbain Sandra L. Haberichter and Veronica H. Flood17 Clinical and molecular markers of VWD2N 149Jenny Goudemand and Mélanie Daniel18 Clinical laboratory and molecular markers of type 3 von Willebrand disease 159Luciano Baronciani and Augusto B. Federici19 Pediatric aspects of von Willebrand disease 177Robert F. Sidonio Jr and Jorge Di Paola20 Women with von Willebrand Disease 185Rezan Abdul Kadir21 The use of desmopressin in von Willebrand disease 201Giancarlo Castaman and Augusto B. Federici22 Plasma-derived and recombinant VWF concentrates 215Pier Mannuccio Mannucci and Massimo Franchini23 Pathophysiology epidemiology and management of acquired von Willebrand syndrome 221Ulrich Budde and Augusto B. Federici24 Cardiovascular causes of AVWS 239Antoine Rauch and Sophie Susen25 Von Willebrand factor regulation of angiogenesis and vascular integrity: implications forgastrointestinal angiodysplasia and beyond 247Anna M. Randi and Giancarlo Castaman26 Prophylaxis in von Willebrand disease 257Erik E. Berntorp and Augusto B. Federici27 Risk of thrombosis and antithrombotic treatment in von Willebrand disease patients 265Ferdows Atiq and Frank W.G. Leebeek28 Novel functions for VWF beyond hemostasis 271Ferdows Atiq and James S. O’DonnellIndex 281

Professor Augusto B. Federici, Hematology and Transfusion Medicine, L. Sacco University Hospital and University of Milan, Milan, Italy. Professor Erik E. Berntorp, Department of Translational Medicine, Malmö Centre for Thrombosis and Haemostasis, Lund University, Skåne University Hospital, Malmö, Sweden. Professor David Lillicrap, Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen’s University, Kingston, ON, Canada. Professor Robert R. Montgomery, Pediatrics (Hematology) and Vice Chair, Versiti Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI, USA.

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• List of Contributors viiForeword xiPreface xiii1 Historical perspective on von Willebrand disease 1Erik E. Berntorp and Margareta Blombäck2 Biosynthesis and organization of von Willebrand factor 7Sandra L. Haberichter3 Von Willebrand factor structure and function 23Robert R. Montgomery Veronica H. Flood and Sandra L. Haberichter4 Regulation of von Willebrand factor expression 39Elham Ghorbanpour and David Lillicrap5 Modulation of VWF by ADAMTS13 49Yaoxian Xu Anastasis Petri and James T.B. Crawley6 Assessment of VWF clearance 63Orla Rawley Laura L. Swystun and Jamie M. O’Sullivan7 Classification of VWD 73Jessica Garcia Sandra L. Haberichter and Veronica H. Flood8 The epidemiology of von Willebrand disease 81Giancarlo Castaman and Francesco Rodeghiero9 Clinical aspects of von Willebrand disease: bleeding history 87Paula D. James and Alberto Tosetto10 Laboratory diagnosis of von Willebrand disease: the phenotype 95Emmanuel J. Favaloro and Ulrich Budde11 Molecular diagnosis of von Willebrand disease: the genotype 109Pamela Christopherson Robert R. Montgomery and Veronica H. Flood12 Clinical laboratory and molecular markers of type 1 von Willebrand disease and low vonWillebrand factor 115David Lillicrap13 Clinical and molecular markers of type 1C VWD 123Heather Clift Veronica H. Flood and Sandra L. Haberichter14 Clinical and molecular markers of VWD2A 129Reinhard Schneppenheim and Giancarlo Castaman15 Clinical and molecular markers of VWD2B 137Giancarlo Castaman and Augusto B. Federici16 Clinical and molecular markers of type 2M VWD 143Maissaa Janbain Sandra L. Haberichter and Veronica H. Flood17 Clinical and molecular markers of VWD2N 149Jenny Goudemand and Mélanie Daniel18 Clinical laboratory and molecular markers of type 3 von Willebrand disease 159Luciano Baronciani and Augusto B. Federici19 Pediatric aspects of von Willebrand disease 177Robert F. Sidonio Jr and Jorge Di Paola20 Women with von Willebrand Disease 185Rezan Abdul Kadir21 The use of desmopressin in von Willebrand disease 201Giancarlo Castaman and Augusto B. Federici22 Plasma-derived and recombinant VWF concentrates 215Pier Mannuccio Mannucci and Massimo Franchini23 Pathophysiology epidemiology and management of acquired von Willebrand syndrome 221Ulrich Budde and Augusto B. Federici24 Cardiovascular causes of AVWS 239Antoine Rauch and Sophie Susen25 Von Willebrand factor regulation of angiogenesis and vascular integrity: implications forgastrointestinal angiodysplasia and beyond 247Anna M. Randi and Giancarlo Castaman26 Prophylaxis in von Willebrand disease 257Erik E. Berntorp and Augusto B. Federici27 Risk of thrombosis and antithrombotic treatment in von Willebrand disease patients 265Ferdows Atiq and Frank W.G. Leebeek28 Novel functions for VWF beyond hemostasis 271Ferdows Atiq and James S. O’DonnellIndex 281

Professor Augusto B. Federici, Hematology and Transfusion Medicine, L. Sacco University Hospital and University of Milan, Milan, Italy. Professor Erik E. Berntorp, Department of Translational Medicine, Malmö Centre for Thrombosis and Haemostasis, Lund University, Skåne University Hospital, Malmö, Sweden. Professor David Lillicrap, Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen’s University, Kingston, ON, Canada. Professor Robert R. Montgomery, Pediatrics (Hematology) and Vice Chair, Versiti Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI, USA.

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• List of Contributors viiForeword xiPreface xiii1 Historical perspective on von Willebrand disease 1Erik E. Berntorp and Margareta Blombäck2 Biosynthesis and organization of von Willebrand factor 7Sandra L. Haberichter3 Von Willebrand factor structure and function 23Robert R. Montgomery Veronica H. Flood and Sandra L. Haberichter4 Regulation of von Willebrand factor expression 39Elham Ghorbanpour and David Lillicrap5 Modulation of VWF by ADAMTS13 49Yaoxian Xu Anastasis Petri and James T.B. Crawley6 Assessment of VWF clearance 63Orla Rawley Laura L. Swystun and Jamie M. O’Sullivan7 Classification of VWD 73Jessica Garcia Sandra L. Haberichter and Veronica H. Flood8 The epidemiology of von Willebrand disease 81Giancarlo Castaman and Francesco Rodeghiero9 Clinical aspects of von Willebrand disease: bleeding history 87Paula D. James and Alberto Tosetto10 Laboratory diagnosis of von Willebrand disease: the phenotype 95Emmanuel J. Favaloro and Ulrich Budde11 Molecular diagnosis of von Willebrand disease: the genotype 109Pamela Christopherson Robert R. Montgomery and Veronica H. Flood12 Clinical laboratory and molecular markers of type 1 von Willebrand disease and low vonWillebrand factor 115David Lillicrap13 Clinical and molecular markers of type 1C VWD 123Heather Clift Veronica H. Flood and Sandra L. Haberichter14 Clinical and molecular markers of VWD2A 129Reinhard Schneppenheim and Giancarlo Castaman15 Clinical and molecular markers of VWD2B 137Giancarlo Castaman and Augusto B. Federici16 Clinical and molecular markers of type 2M VWD 143Maissaa Janbain Sandra L. Haberichter and Veronica H. Flood17 Clinical and molecular markers of VWD2N 149Jenny Goudemand and Mélanie Daniel18 Clinical laboratory and molecular markers of type 3 von Willebrand disease 159Luciano Baronciani and Augusto B. Federici19 Pediatric aspects of von Willebrand disease 177Robert F. Sidonio Jr and Jorge Di Paola20 Women with von Willebrand Disease 185Rezan Abdul Kadir21 The use of desmopressin in von Willebrand disease 201Giancarlo Castaman and Augusto B. Federici22 Plasma-derived and recombinant VWF concentrates 215Pier Mannuccio Mannucci and Massimo Franchini23 Pathophysiology epidemiology and management of acquired von Willebrand syndrome 221Ulrich Budde and Augusto B. Federici24 Cardiovascular causes of AVWS 239Antoine Rauch and Sophie Susen25 Von Willebrand factor regulation of angiogenesis and vascular integrity: implications forgastrointestinal angiodysplasia and beyond 247Anna M. Randi and Giancarlo Castaman26 Prophylaxis in von Willebrand disease 257Erik E. Berntorp and Augusto B. Federici27 Risk of thrombosis and antithrombotic treatment in von Willebrand disease patients 265Ferdows Atiq and Frank W.G. Leebeek28 Novel functions for VWF beyond hemostasis 271Ferdows Atiq and James S. O’DonnellIndex 281