Diagnosis and Management of von Willebrand Disease in Spain

Javier Batlle; Almudena Perez-Rodriguez; Joana Costa Pinto; Esther Loures Fraga N.R.; Angela Rodriguez-Trillo Tch; Maria Fernanda Lopez-Fernandez

doi:10.1055/s-0031-1281036

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2011; 37(5): 503-510
DOI: 10.1055/s-0031-1281036

Diagnosis and Management of von Willebrand Disease in Spain

Javier Batlle¹ ^, ² , Almudena Perez-Rodriguez¹ , Joana Costa Pinto¹ , Esther Loures Fraga N.R.¹ , Angela Rodriguez-Trillo Tch¹ , Maria Fernanda Lopez-Fernandez¹ on behalf of participant investigators and centers*

¹Haematology and Haemotherapy Service and INIBIC, Complexo Hospitalario Universitario de A Coruna, A Coruna, Spain
²Department of Medicine, University of Santiago de Compostela, A Coruña, Spain

Abstract

ABSTRACT

The correct diagnosis and classification of von Willebrand disease (VWD) is important for therapy and genetic counseling but is made difficult due to the variability of its clinical expression and limitations of laboratory methods. A national registry of VWD patients has been initiated in Spain. The results of a concise survey on the diagnosis of VWD show the frequency of VWD is fivefold greater in Spain than that expected from epidemiological studies in other European countries; this may result from overdiagnosis and/or a higher prevalence of VWD. These results clearly reinforce the need for the Spanish VWD registry. A consensus guideline for optimal treatment of VWD is being elaborated in Spain. Desmopressin (DDAVP) is the choice of treatment in responsive VWD patients. von Willebrand factor concentrates (VWF/factor VIII) are used in individuals nonresponsive to DDAVP, when DDAVP is contraindicated, or in VWD types 2B and 3.

KEYWORDS

von Willebrand disease - VWD - von Willebrand factor - VWF - survey - diagnosis - therapy

Full Text

References

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Javier BatlleMD

Department of Medicine, University of Santiago de Compostela

Spain. A Coruna (Hospital Materno Infantil building). Carretera del Pasaje s/n. 15006 - A Coruña. España

Email: Francisco.Javier.Batlle.Fonrodona@sergas.es