Evidence-based recommendations on the treatment of von Willebrand disease in Italy
Related Experiment Video
Updated: Jun 22, 2026

Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
Published on: August 14, 2017
Pier Mannuccio Mannucci1, Massimo Franchini, Giancarlo Castaman
1Centro Emofilia e Trombosi Angelo Bianchi Bonomi, Dipartimento di Medicina e Specialità Mediche, Università di Milano e IRCCS Fondazione Ospedale Maggiore, Italy. piermannuccio.mannucci@unimi.it
Background:
von Willebrand disease (VWD) is the most common hereditary bleeding disorder affecting both males and females. It arises from quantitative or qualitative defects of von Willebrand factor (VWF) and causes bleeding of mucous membranes and soft tissues. The aim of treatment is to correct the dual defect of haemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII).
Material And Methods:
This document contains evidence-based recommendations for the management of VWD compiled by AICE (the Italian Association of Haemophilia Centres). All the evidence supporting these recommendations are based on non-randomised comparative studies or case series, because randomised controlled clinical trials or meta-analyses are not available for this disease.
Results And Conclusions:
Desmopressin (DDAVP) is the treatment of choice for patients with type 1 VWD with FVIII and VWF levels of 10 U/dL or more, while VWF/FVIII concentrates are indicated for those who are unresponsive or insufficiently responsive to DDAVP (severe type 1, type 2 and 3 VWD). VWF concentrates devoid of FVIII, not yet licensed in Italy, may be considered for short-term prophylaxis in elective surgery or for long-term secondary prophylaxis.
Related Concept Videos
Venous Thrombosis III: Interprofessional Care
Varicose Veins II: Diagnostic Studies and Interprofessional Care
Rheumatic Heart Disease III: Medical Management
Esophageal Varices-II: Clinical Features and Management
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol abuse, or...
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Mitral Stenosis III: Medical Management
