Textbook of Von Willebrand Disease - Basic and Clinical Aspects, 2e (Mar 25, 2024)_(1119419484)_(Wiley-Blackwell).pdf

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. Cover Title Page Copyright Page Contents List of Contributors Foreword Preface Chapter 1 Historical perspective on von Willebrand disease Introduction The scientist of the disease First description of the disease: the Åland family Other early clinical reports The search for a new factor—the bleeding time factor The end of the beginning Recent scientific visits to Åland Islands References Chapter 2 Biosynthesis and organization of von Willebrand factor Introduction Terminology Molecular biology of VWF The VWF gene VWF domain structure VWF promoter Cell biology of VWF VWF processing and dimerization in the endoplasmic reticulum VWF processing in the Golgi VWF multimerization Regulated storage of VWF in Weibel–Palade bodies and -granules Weibel–Palade body biogenesis Regulated release of VWF Postsecretion modification of VWF References Chapter 3 Von Willebrand factor structure and function Introduction VWF function Structure of VWF VWF domain structure Functional biochemistr