Titre
Evidence-based recommendations on the treatment of von Willebrand disease in Italy.
Type
article
Institution
Externe
Périodique
Blood Transfusion = Trasfusione Del Sangue
Auteur(s)
Mannucci, P.M.
Auteure/Auteur
Franchini, M.
Auteure/Auteur
Castaman, G.
Auteure/Auteur
Federici, A.B.
Auteure/Auteur
Contributrices/contributeurs
Contino, L.
Accorsi, A.
Ciavarella, N.
Schiavoni, M.
Scaraggi, FA.
Rodorigo, G.
Valdre, L.
Targhetta, R.
Tagariello, G.
Radossi, P.
Musso, R.
Cultrera, D.
Muleo, G.
Iannacaro, P.
Biasioli, C.
Testa, S.
Alatri, A.
Vincenzi, D.
Scapoli, G.
Morfini, M.
Molinari, AC.
Boeri, E.
Caprino, D.
Delios, G.
Girotto, M.
Mariani, G.
Lapecorella, M.
Carloni, MT.
Cantori, I.
Santagostino, E.
Gringeri, A.
Marietta, M.
Pedrazzoli, P.
Di Minno, G.
Coppola, A.
Perricone, C.
Schiavulli, M.
Rocino, A.
Berrettini, M.
Zanon, E.
Mancuso, G.
Siragusa, S.
Malato, A.
Saccullo, G.
Tagliaferri, A.
Rivolta, F.
Iorio, A.
Oliovecchio, E.
Ferrante, F.
Dragani, A.
Rossi, A.
Mancino, A.
Albertini, P.
Macchi, S.
D'Incà, M.
De Rossi, G.
Luciani, M.
Landolfi, R.
Mazzucconi, MG.
Santoro, C.
Piseddu, G.
Schinco, PC.
Rossetti, G.
Barillari, G.
Feola, G.
Gandini, G.
Groupes de travail
Italian Association of Hemophilia Centers
Liens vers les personnes
ISSN
1723-2007
Statut éditorial
Publié
Date de publication
2009
Volume
7
Numéro
2
Première page
117
Dernière page/numéro d’article
126
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Practice Guideline
Résumé
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.
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.
PID Serval
serval:BIB_4BBCE8D8B7A7
PMID
Date de création
2013-02-14T12:36:26.734Z
Date de création dans IRIS
2025-05-20T17:06:22Z