Meeting Report

Blood Transfusion - 4 2018 (July - August)

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

Authors

Publication Date: 2017-05-26

Abstract


Introduction
Von Willebrand's disease (vWD) is a rare inherited bleeding disorder (Orphanet number: ORPHA903) caused by the quantitative or qualitative deficiency of von Willebrand factor (vWF)1. The clinical hallmark of vWD is mucocutaneous bleeding (e.g. epistaxis, menorrhagia and gastrointestinal bleeding), but in severe forms, in which factor VIII (FVIII) levels are also significantly reduced, spontaneous haemarthroses or haematomas may occur. The severity of bleeding phenotype is still not well defined within different types of vWD2,3, and yet this is actually the key factor driving treatment decisions. As the bleeding pattern can affect quality of life and even survival3-7, it is essential to optimise the diagnostic process in order to ensure the most adequate therapeutic strategy promptly in patients presenting with spontaneous bleeding. Unfortunately, in several Centres worldwide the correct clinical and laboratory diagnosis is still hampered by the marked heterogeneity of vWD phenotypes and the need for multiple, sometimes technically complex, tests. [...]

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Authors

Piercarla Schinco - RRC Thrombosis & Haemophilia Centre, AOU "Città della Salute e della Scienza" - Molinette Hospital, Turin

Giancarlo Castaman - Centre for Bleeding Disorders, Careggi University Hospital, Florence

Antonio Coppola - Regional Reference Centre for Coagulation Disorders, "Federico II" University Hospital, Naples

Dorina Cultrera - Department of Haematology, Haemophilia Regional Reference Centre, University Hospital of Catania, Catania

Cosimo Ettorre - Haemophilia and Thrombosis Centre, "Policlinico Giovanni XXIII", Bari

Anna C. Giuffrida - Haemophilia Centre, Blood Transfusion Centre, University of Verona, Verona

Emanuela Marchesini - Department of Vascular and Emergency Medicine, "Santa Maria della Misericordia" University Hospital, Perugia

Renato Marino - Haemophilia and Thrombosis Centre, "Policlinico Giovanni XXIII", Bari

Marta Milan - Department of Cardiac Thoracic and Vascular Sciences, Haemophilia Centre Unit of Coagulopathies, University Hospital of Padua, Padua

Claudio Molinari - Thrombosis and Haemostasis Unit, "Giannina Gaslini" Children's Hospital, Genoa

Simona M. Siboni - "Angelo Bianchi Bonomi" Haemophilia and Thrombosis Centre, "Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano", Milan

Ezio Zanon - Department of Cardiac Thoracic and Vascular Sciences, Haemophilia Centre Unit of Coagulopathies, University Hospital of Padua, Padua

Augusto B. Federici - Division of Haematology and Transfusion Medicine, "L. Sacco" University Hospital and Department of Oncology and Haematology Oncology, University of Milan, Milan, Italy

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