Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Practice Guideline
. 2009 Apr;7(2):117-26.
doi: 10.2450/2008.0052-08.

Evidence-based recommendations on the treatment of von Willebrand disease in Italy

Pier Mannuccio Mannucci  1 , Massimo Franchini, Giancarlo Castaman, Augusto B Federici; Italian Association of Hemophilia Centers
Collaborators, Affiliations
Practice Guideline

Evidence-based recommendations on the treatment of von Willebrand disease in Italy

Pier Mannuccio Mannucci et al. Blood Transfus. 2009 Apr.

Abstract

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.

Keywords: desmopressin; von Willebrand disease; von Willebrand factor.

PubMed Disclaimer

References

    1. Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand’s disease. Blood. 1987;69:454–9. - PubMed
    1. Castaman G, Federici AB, Rodeghiero F, Mannucci PM. von Willebrand’s disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica. 2003;88:94–108. - PubMed
    1. Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand factor. J Thromb Haemost. 2006;4:2103–14. - PubMed
    1. Mannucci PM. How I treat patients with von Willebrand disease. Blood. 2001;97:1915–9. - PubMed
    1. Mannucci PM. Treatment of von Willebrand’s disease. N Engl J Med. 2004;351:683–94. - PubMed

Publication types