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

Piercarla Schinco, Giancarlo Castaman, Antonio Coppola, Dorina Cultrera, Cosimo Ettorre, Anna C. Giuffrida, Emanuela Marchesini, Renato Marino, Marta Milan, Claudio Molinari, Simona M. Siboni, Ezio Zanon, Augusto B. Federici

Research output: Contribution to journalArticle

3 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)371-381
Number of pages11
JournalBlood Transfusion
Volume16
Issue number4
DOIs
Publication statusPublished - Jul 1 2018

Fingerprint

von Willebrand Diseases
Italy
von Willebrand Factor
Enalaprilat
Workflow
Factor VIII
Physiologic Monitoring
Guidelines
Hemorrhage
Safety

Keywords

  • Management
  • Secondary long-term and surgical prophylaxis
  • Von Willebrand factor-containing concentrates
  • Von Willebrand's disease

ASJC Scopus subject areas

  • Immunology and Allergy
  • Hematology

Cite this

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. / Schinco, Piercarla; Castaman, Giancarlo; Coppola, Antonio; Cultrera, Dorina; Ettorre, Cosimo; Giuffrida, Anna C.; Marchesini, Emanuela; Marino, Renato; Milan, Marta; Molinari, Claudio; Siboni, Simona M.; Zanon, Ezio; Federici, Augusto B.

In: Blood Transfusion, Vol. 16, No. 4, 01.07.2018, p. 371-381.

Research output: Contribution to journalArticle

Schinco, P, Castaman, G, Coppola, A, Cultrera, D, Ettorre, C, Giuffrida, AC, Marchesini, E, Marino, R, Milan, M, Molinari, C, Siboni, SM, Zanon, E & Federici, AB 2018, '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', Blood Transfusion, vol. 16, no. 4, pp. 371-381. https://doi.org/10.2450/2017.0354-16
Schinco, Piercarla ; Castaman, Giancarlo ; Coppola, Antonio ; Cultrera, Dorina ; Ettorre, Cosimo ; Giuffrida, Anna C. ; Marchesini, Emanuela ; Marino, Renato ; Milan, Marta ; Molinari, Claudio ; Siboni, Simona M. ; Zanon, Ezio ; Federici, Augusto B. / 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. In: Blood Transfusion. 2018 ; Vol. 16, No. 4. pp. 371-381.
@article{a3d65acf66cb425bae130a8027c0993e,
title = "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",
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.",
keywords = "Management, Secondary long-term and surgical prophylaxis, Von Willebrand factor-containing concentrates, Von Willebrand's disease",
author = "Piercarla Schinco and Giancarlo Castaman and Antonio Coppola and Dorina Cultrera and Cosimo Ettorre and Giuffrida, {Anna C.} and Emanuela Marchesini and Renato Marino and Marta Milan and Claudio Molinari and Siboni, {Simona M.} and Ezio Zanon and Federici, {Augusto B.}",
year = "2018",
month = "7",
day = "1",
doi = "10.2450/2017.0354-16",
language = "English",
volume = "16",
pages = "371--381",
journal = "Blood Transfusion",
issn = "1723-2007",
publisher = "Edizioni SIMTI",
number = "4",

}

TY - JOUR

T1 - Current challenges in the diagnosis and management of patients with inherited von Willebrand's disease in Italy

T2 - An Expert Meeting Report on the diagnosis and surgical and secondary long-term prophylaxis

AU - Schinco, Piercarla

AU - Castaman, Giancarlo

AU - Coppola, Antonio

AU - Cultrera, Dorina

AU - Ettorre, Cosimo

AU - Giuffrida, Anna C.

AU - Marchesini, Emanuela

AU - Marino, Renato

AU - Milan, Marta

AU - Molinari, Claudio

AU - Siboni, Simona M.

AU - Zanon, Ezio

AU - Federici, Augusto B.

PY - 2018/7/1

Y1 - 2018/7/1

N2 - 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.

AB - 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.

KW - Management

KW - Secondary long-term and surgical prophylaxis

KW - Von Willebrand factor-containing concentrates

KW - Von Willebrand's disease

UR - http://www.scopus.com/inward/record.url?scp=85047967291&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85047967291&partnerID=8YFLogxK

U2 - 10.2450/2017.0354-16

DO - 10.2450/2017.0354-16

M3 - Article

AN - SCOPUS:85047967291

VL - 16

SP - 371

EP - 381

JO - Blood Transfusion

JF - Blood Transfusion

SN - 1723-2007

IS - 4

ER -