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

Pier O. Mannucci, Massimo Franchini, Giancarlo Castaman, Augusto B. Federici

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)117-126
Number of pages10
JournalBlood Transfusion
Volume7
Issue number2
DOIs
Publication statusPublished - 2009

Keywords

  • Desmopressin
  • Von Willebrand disease
  • Von Willebrand factor

ASJC Scopus subject areas

  • Hematology
  • Immunology and Allergy

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