Descarga: Treatment of von Willebrand Disease
Archivo: eha/vonwill/STH 2016 treatment of VWD.pdf
Congenital von Willebrand disease (VWD) and acquired von Willebrand syndrome (AVWS) reflect conditions caused by von Willebrand factor (VWF) deficiency and/or defects.VWDisthemostcommoninheritedbleedingdisorderandAVWSarisesfroma variety of causes. Since VWF stabilizes and protects factor VIII (FVIII) in the circulation, this is also reduced in many patients with VWD. The treatment of VWD and AVWS thereforeprimarilyentailsreplacementofVWF,andsometimesFVIII,to protectagainst bleeding. This may entail the use of VWF concentrates (currently plasma-derived) and/ or FVIII concentrates (currently plasma-derived or more increasingly recombinant forms), and/or desmopressin to release endogenous VWF in subgroups of patients. For AVWS additional treatment of the underlying condition is also required. Adjunct therapiesincludeantifibrinolytics.Globally,variousformulationsexistforbothVWFand FVIII concentrates and are differentially available based on manufacturer marketing or regulatoryapprovals/clearancesindifferentgeographies.Also,guidelinesfortreatment of VWD vary for different localities and recombinant VWF is undergoing clinical trials. ThecurrentreviewprovidesanoverviewofthetreatmentofVWDascurrentlypracticed in developed countries, and also provides a glimpse towards the future.
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