Replacement therapy is a treatment that replaces something that’s missing or not working well in your body. People with von Willebrand’s disease don’t have von Willebrand factor, or it doesn’t work well enough to help clot their blood. This disease can be treated by injecting (infusing) a purified form of clotting factors into a vein. These clotting factors include von Willebrand factor and factor VIII. Infusions of clotting factors help blood to clot normally.
Replacement therapy injections (also called infusions) may be used with people who:
Do not respond to desmopressin (DDAVP, Stimate).
Cannot tolerate desmopressin.
Have severe type 2 or type 3 von Willebrand’s disease.
Have severe bleeding, from an injury or an unknown cause.
You may get this medicine in a hospital or take it at home. Patients often learn to inject themselves at home. But a doctor or nurse can give the shots too.
Replacement therapy doesn’t always work well, because the body may stop the newly introduced von Willebrand factor from working.
Hillman R, et al. (2011). Platelet dysfunction and von Willebrand disease. In Hematology in Clinical Practice, 5th ed., pp. 384-397. New York: McGraw-Hill.
National Heart, Lung, and Blood Institute (2007). The Diagnosis, Evaluation, and Management of von Willebrand Disease. (NIH Publication No. 08-5832). Available online: http://www.nhlbi.nih.gov/guidelines/vwd.
Nichols WL (2016). Von Willebrand disease and hemorrhagic abnormalities of platelet and vascular function. In L Goldman, A Shafer, eds., Goldman-Cecil Medicine, 24th ed., vol. 1, pp. 1167-1172. Philadelphia: Saunders.
ByHealthwise Staff Primary Medical ReviewerE. Gregory Thompson, MD – Internal Medicine Adam Husney, MD – Family Medicine Specialist Medical ReviewerBrian Leber, MDCM, FRCPC – Hematology