Recent advances in the care of von Willebrand's disease (vWD) have allowed the majority of patients to be managed adequately. Even in the more severe forms, it is now possible to control recurrent bleeding through secondary long-term prophylaxis with von Willebrand factor-containing concentrates. Moreover, in the setting of surgical prophylaxis, the combination of interdisciplinary management and close patient monitoring yields a positive outcome in nearly all cases, although safety concerns remain. In clinical practice, the effectiveness of therapy is hindered by the difficulties in making a rapid, yet accurate diagnosis, in identifying the subgroup of bleeders who may benefit most from a specific strategy, and in selecting the optimal product and regimen. Since specific guidelines for heavy bleeders requiring short- and long-term prophylaxis are still lacking, sharing the experience of experts dealing with vWD patients on a daily basis is crucial to fill gaps in information relating to patient management. To address this important issue, 13 Italian haematologists met in Milan on April, 2, 2016 and in Florence on July, 9, 2016. A 30-question survey constituted the input to discuss (i) optimisation of the diagnostic workflow for vWD, (ii) the characteristics of patients who may benefit from secondary long-term prophylaxis (in particular with the purified von Willebrand factor concentrate with a low content of factor VIII), (iii) the key elements to consider when selecting a concentrate and (iv) the pre-operative and post-operative management of vWD patients. A summary of the main points covered is provided in this report.

Current challenges in the diagnosis and management of patients with inherited von Willebrand's disease in Italy: An Expert Meeting Report on the diagnosis and surgical and secondary long-term prophylaxis / P. Schinco, G. Castaman, A. Coppola, D. Cultrera, C. Ettorre, A.C. Giuffrida, E. Marchesini, R. Marino, M. Milan, C. Molinari, S.M. Siboni, E. Zanon, A.B. Federici. - In: BLOOD TRANSFUSION. - ISSN 1723-2007. - 16:4(2018), pp. 371-381. [10.2450/2017.0354-16]

Current challenges in the diagnosis and management of patients with inherited von Willebrand's disease in Italy: An Expert Meeting Report on the diagnosis and surgical and secondary long-term prophylaxis

G. Castaman
Secondo
;
S.M. Siboni;A.B. Federici
Ultimo
2018

Abstract

Recent advances in the care of von Willebrand's disease (vWD) have allowed the majority of patients to be managed adequately. Even in the more severe forms, it is now possible to control recurrent bleeding through secondary long-term prophylaxis with von Willebrand factor-containing concentrates. Moreover, in the setting of surgical prophylaxis, the combination of interdisciplinary management and close patient monitoring yields a positive outcome in nearly all cases, although safety concerns remain. In clinical practice, the effectiveness of therapy is hindered by the difficulties in making a rapid, yet accurate diagnosis, in identifying the subgroup of bleeders who may benefit most from a specific strategy, and in selecting the optimal product and regimen. Since specific guidelines for heavy bleeders requiring short- and long-term prophylaxis are still lacking, sharing the experience of experts dealing with vWD patients on a daily basis is crucial to fill gaps in information relating to patient management. To address this important issue, 13 Italian haematologists met in Milan on April, 2, 2016 and in Florence on July, 9, 2016. A 30-question survey constituted the input to discuss (i) optimisation of the diagnostic workflow for vWD, (ii) the characteristics of patients who may benefit from secondary long-term prophylaxis (in particular with the purified von Willebrand factor concentrate with a low content of factor VIII), (iii) the key elements to consider when selecting a concentrate and (iv) the pre-operative and post-operative management of vWD patients. A summary of the main points covered is provided in this report.
von Willebrand's disease; secondary long-term and surgical prophylaxis; von Willebrand factor-containing concentrates; management
Settore MED/15 - Malattie del Sangue
26-mag-2017
Article (author)
File in questo prodotto:
File Dimensione Formato  
Blood Transfus 2018.pdf

accesso riservato

Tipologia: Publisher's version/PDF
Dimensione 1.39 MB
Formato Adobe PDF
1.39 MB Adobe PDF   Visualizza/Apri   Richiedi una copia
Pubblicazioni consigliate

Caricamento pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/667087
Citazioni
  • ???jsp.display-item.citation.pmc??? 1
  • Scopus 9
  • ???jsp.display-item.citation.isi??? 10
social impact