title: State of the art: von Willebrand disease.
abstract: The State of the Art in von Willebrand disease (VWD) has been impacted not only by discoveries in the field of haemostasis, but also by changes in practice in other fields. The development of bleeding assessment tools has led to the clarification of bleeding symptoms and phenotype in VWD. New discoveries in the biology and genetics of von Willebrand factor (VWF) are challenging our existing diagnostics and classification(s). An improved understanding of reproductive physiology and the pathology of VWD along with changing obstetric, gynaecologic and haemostatic therapies necessitate an evolving response to the care of women with VWD. The survival of patients with autoimmune disease, malignancies and congenital heart disease along with increasing use of circulatory support devices and extracorporeal membrane oxygenation is increasing the prevalence of acquired von Willebrand syndrome. In each of these challenges, there are opportunities to improve the care of our patients with VWD.
authors.affiliation: Division of Maternal-Fetal Medicine, Department of Obstetrics & Gynecology, Duke University Medical Center, Durham, NC, USA.|Division of Hematology, Department of Medicine, Duke University, Medical Center, Durham, NC, USA.
authors.forename: A H
authors.initials: AH
authors.lastname: James
authors.affiliation: Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Eikenboom
authors.affiliation: Department of Oncology and Hematology-Oncology, Hematology and Transfusion Medicine, L. Sacco University Hospital, University of Milan, Milan, Italy.
authors.forename: A B
authors.initials: AB
authors.lastname: Federici
mesh_terms: D057134:Antibodies, Neutralizing / Q000097:blood; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D011110:Polymorphism, Genetic; D006473:Postpartum Hemorrhage / Q000517:prevention & control; D011247:Pregnancy; D011994:Recombinant Proteins / Q000627:therapeutic use; D014842:von Willebrand Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D014841:von Willebrand Factor / Q000032:analysis* / Q000627:therapeutic use
chemical_list: D057134:Antibodies, Neutralizing; D011994:Recombinant Proteins; D014841:von Willebrand Factor; C103587:recombinant FVIIa; D015942:Factor VIIa; D003894:Deamino Arginine Vasopressin
keywords: acquired von Willebrand syndrome; childbirth; diagnostic assays; heavy menstrual bleeding; laboratory assessment; von Willebrand disease