The Use of Plasma-Derived Concentrates

Pier Mannuccio Mannucci; Massimo Franchini

doi:10.1002/9781444329926.ch17

The Use of Plasma-Derived Concentrates

Pier Mannuccio Mannucci, Massimo Franchini

Fondazione IRCCS Ca' Granda- Ospedale Maggiore Policlinico

Research output: Chapter in Book/Report/Conference proceeding › Chapter

Abstract

Von Willebrand disease (VWD) is the most common hereditary bleeding disorder that affects both males and females. It arises from quantitative or qualitative defects of von Willebrand factor (VWF) and causes mucous membrane and soft-tissue bleeding. The aim of treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced VWF and the concomitant secondary deficiency of factor VIII (FVIII). Desmopressin (DDAVP) is the treatment of choice for patients with type 1 VWD who have FVIII and VWF levels of 10 IU/dL or more, while plasma-derived VWF concentrates, either containing FVIII or devoid of this moiety, are indicated for those who are unresponsive or insufficiently responsive to DDAVP (severe type 1, type 2, and type 3 VWD).

Original language	English
Title of host publication	Von Willebrand Disease: Basic and Clinical Aspects
Publisher	Wiley-Blackwell
Pages	200-206
Number of pages	7
ISBN (Print)	9781405195126
DOIs	https://doi.org/10.1002/9781444329926.ch17
Publication status	Published - Mar 21 2011

Keywords

Bleeding
Factor VIII
Therapy
Thrombosis
Von Willebrand disease
Von Willebrand factor
VWF/FVIII concentrates

ASJC Scopus subject areas

Medicine(all)

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10.1002/9781444329926.ch17

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abstract = "Von Willebrand disease (VWD) is the most common hereditary bleeding disorder that affects both males and females. It arises from quantitative or qualitative defects of von Willebrand factor (VWF) and causes mucous membrane and soft-tissue bleeding. The aim of treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced VWF and the concomitant secondary deficiency of factor VIII (FVIII). Desmopressin (DDAVP) is the treatment of choice for patients with type 1 VWD who have FVIII and VWF levels of 10 IU/dL or more, while plasma-derived VWF concentrates, either containing FVIII or devoid of this moiety, are indicated for those who are unresponsive or insufficiently responsive to DDAVP (severe type 1, type 2, and type 3 VWD).",

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