Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A

Giancarlo Castaman, Silvia Linari Department of Oncology, Center for Bleeding Disorders, Careggi University Hospital, Florence, ItalyAbstract: Several plasma-derived intermediate and high-purity concentrates containing von Willebrand factor (VWF) and factor VIII (FVIII) are currently available. The...

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Main Authors: Castaman G, Linari S
Format: Article
Language:English
Published: Dove Medical Press 2016-06-01
Series:Therapeutics and Clinical Risk Management
Subjects:
Online Access:https://www.dovepress.com/human-von-willebrand-factorfactor-viii-concentrates-in-the-management--peer-reviewed-article-TCRM
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author Castaman G
Linari S
author_facet Castaman G
Linari S
author_sort Castaman G
collection DOAJ
description Giancarlo Castaman, Silvia Linari Department of Oncology, Center for Bleeding Disorders, Careggi University Hospital, Florence, ItalyAbstract: Several plasma-derived intermediate and high-purity concentrates containing von Willebrand factor (VWF) and factor VIII (FVIII) are currently available. The main role of these products in the management of pediatric the population is represented by the replacement therapy in patients with severe or intermediate forms of von Willebrand disease, in whom other treatments are ineffective or contraindicated. Another important role of VWF/FVIII concentrates in children may be their use in immune tolerance induction (ITI) protocols. ITI is particularly recommended for hemophilia A children who have developed an inhibitor against FVIII, currently the most serious complication of substitutive treatment in hemophilia. Although recombinant concentrates may represent the preferred option in children with hemophilia A, VWF/FVIII concentrates may offer an advantage in rescuing patients who failed previous ITI.Keywords: von Willebrand factor, factor VIII, plasma-derived concentrates, children, von Willebrand disease, hemophilia A
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spelling doaj.art-57aca2fd779b4b4eabd54cb7234f269d2022-12-21T19:30:04ZengDove Medical PressTherapeutics and Clinical Risk Management1178-203X2016-06-012016Issue 11029103727724Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia ACastaman GLinari SGiancarlo Castaman, Silvia Linari Department of Oncology, Center for Bleeding Disorders, Careggi University Hospital, Florence, ItalyAbstract: Several plasma-derived intermediate and high-purity concentrates containing von Willebrand factor (VWF) and factor VIII (FVIII) are currently available. The main role of these products in the management of pediatric the population is represented by the replacement therapy in patients with severe or intermediate forms of von Willebrand disease, in whom other treatments are ineffective or contraindicated. Another important role of VWF/FVIII concentrates in children may be their use in immune tolerance induction (ITI) protocols. ITI is particularly recommended for hemophilia A children who have developed an inhibitor against FVIII, currently the most serious complication of substitutive treatment in hemophilia. Although recombinant concentrates may represent the preferred option in children with hemophilia A, VWF/FVIII concentrates may offer an advantage in rescuing patients who failed previous ITI.Keywords: von Willebrand factor, factor VIII, plasma-derived concentrates, children, von Willebrand disease, hemophilia Ahttps://www.dovepress.com/human-von-willebrand-factorfactor-viii-concentrates-in-the-management--peer-reviewed-article-TCRMvon Willebrand factorfactor VIIIplasma-derived concentrateschildrenvon Willebrand diseasehemophilia A
spellingShingle Castaman G
Linari S
Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A
Therapeutics and Clinical Risk Management
von Willebrand factor
factor VIII
plasma-derived concentrates
children
von Willebrand disease
hemophilia A
title Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A
title_full Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A
title_fullStr Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A
title_full_unstemmed Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A
title_short Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A
title_sort human von willebrand factor factor viii concentrates in the management of pediatric patients with von willebrand disease hemophilia a
topic von Willebrand factor
factor VIII
plasma-derived concentrates
children
von Willebrand disease
hemophilia A
url https://www.dovepress.com/human-von-willebrand-factorfactor-viii-concentrates-in-the-management--peer-reviewed-article-TCRM
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