Slovenský hematolologický portál - Treatment of patients with von Willebrand disease

Treatment of patients with von Willebrand disease

Emma Tuohy1, Emma Litt1, Raza Alikhan1,2
1Department of Haematology, University Hospital of Wales, Cardiff, UK
2Haemophilia and Thrombosis Centre, University Hospital of Wales, Cardiff, UK

Published: Journal of Blood Medicine, April 2011 , Volume 2011:2 Pages 49 - 57

Abstract
Von Willebrand disease (vWD) is the most common hereditary bleeding disorder. The aim of therapy is to correct the dual hemostatic defect, due to defective platelet adhesion-aggregation and abnormal coagulation due to Factor VIII (FVIII) deficiency. The choice of treatment depends on a number of factors, including the severity of the bleed, the procedure planned, the subtype and severity of the disease and the age and morbidity of the patient. Desmopressin (DDAVP) is the treatment of choice for type 1 vWD as it increases endogenous release of FVIII and von Willebrand factor (vWF) and is also used in some subtypes of type 2 vWD. In those patients in whom DDAVP is ineffective or contraindicated, levels can be restored by infusing vWF:FVIII concentrates. The role of antifibrinolytic treatment is an important adjunct to replacement therapy during minor or major surgery involving mucosal surfaces. The dosing and timing of vWF:FVIII concentrates is important depending on the nature of the surgical procedure. The role of secondary prophylaxis needs to be further defined.

Keywords: von Willebrand disease, treatment, DDAVP

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