Treatment of von Willebrand Disease

Seminars in Thrombosis and Hemostasis
Jennifer CurnowEmmanuel J Favaloro

Abstract

Congenital von Willebrand disease (VWD) and acquired von Willebrand syndrome (AVWS) reflect conditions caused by von Willebrand factor (VWF) deficiency and/or defects. VWD is the most common inherited bleeding disorder and AVWS arises from a 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 therefore primarily entails replacement of VWF, and sometimes FVIII, to protect against 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 therapies include antifibrinolytics. Globally, various formulations exist for both VWF and FVIII concentrates and are differentially available based on manufacturer marketing or regulatory approvals/clearances in different geographies. Also, guidelines for treatment of VWD vary for different localities and recombinant VWF is undergoing clinical trials. The current review provides an overview of the treatment of V...Continue Reading

Citations

Apr 1, 2016·Haemophilia : the Official Journal of the World Federation of Hemophilia·E J FavaloroK Marsden
May 13, 2017·Haemophilia : the Official Journal of the World Federation of Hemophilia·E J Favaloro, S Oliver
Jan 11, 2017·Journal of Blood Transfusion·Jason P AckerWilliam P Sheffield
May 2, 2018·Clinical Chemistry and Laboratory Medicine : CCLM·Emmanuel J Favaloro, Giuseppe Lippi
Feb 20, 2018·Clinical Chemistry and Laboratory Medicine : CCLM·Giuseppe Lippi, Emmanuel J Favaloro
Mar 30, 2019·The Medical Journal of Australia·Scott DunkleyJennifer L Curnow
Sep 12, 2019·International Journal of Laboratory Hematology·Susan OliverEmmanuel J Favaloro
Jul 31, 2020·Advances in Therapy·Alan D KayeElyse M Cornett
Aug 23, 2017·Expert Review of Hematology·Anne-Mette Hvas, Emmanuel J Favaloro
Jun 27, 2020·Hämostaseologie·Emmanuel J Favaloro
Nov 21, 2020·Haemophilia : the Official Journal of the World Federation of Hemophilia·Emmanuel J FavaloroJennifer Curnow
Feb 27, 2021·Seminars in Thrombosis and Hemostasis·Valder R ArrudaBenjamin J Samelson-Jones
Apr 17, 2021·Experimental Biology and Medicine·Nina Moiseiwitsch, Ashley C Brown
Oct 1, 2021·The Journal of International Medical Research·R Haridian Sosa BarriosMaite Rivera-Gorrin

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