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Von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease.
Blood Coagul Fibrinolysis 2009; 20(2):89-100BC

Abstract

Therapy for von Willebrand disease (VWD) aims to restore the hemostatic function conferred by von Willebrand factor (VWF), which facilitates platelet adhesion and aggregation, and serves to increase potentially low coagulation factor VIII (FVIII) in plasma. In patients unresponsive to desmopressin (DDAVP), the preferred treatment is with plasma-derived VWF-containing FVIII concentrates. Only a few of the available VWF/FVIII concentrates have been licensed for use in VWD based on prospective studies. The efficacy of VWF/FVIII concentrates depends on the content and quality of VWF and FVIII. Several studies have demonstrated the variability of the VWF contents, as well as the differences in the VWF multimer patterns (including the high molecular weight VWF multimers that are most effective in restoring hemostasis), among these concentrates. Treating physicians should be aware of these disparities and the potential clinical implications for patients with different VWD subtypes. Dosing has traditionally been calculated based on the FVIII content of the products, although dosing based on VWF functional activity [e.g., VWF ristocetin cofactor activity (VWF:RCo)] addresses the primary protein deficiency in VWD patients. Several clinical studies have demonstrated the efficacy of concentrates dosed according to VWF:RCo. Dosing is generally consistent across VWD subtypes, although patients with severe phenotypes or undergoing major procedures may require more infusions or longer treatment duration. Other considerations for the use of VWF-containing concentrates include laboratory monitoring of efficacy and safety issues such as thrombosis risk and thromboprophylaxis.

Authors+Show Affiliations

Servicio de Hematología y Hemoterapia, Complexo Hospitalario Universitario Juan Canalejo, Departamento de Medicina, Universidad de Santiago de Compostela, A Corunna, Spain. jbatlle@canalejo.orgNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

19786936

Citation

Batlle, Javier, et al. "Von Willebrand Factor/factor VIII Concentrates in the Treatment of Von Willebrand Disease." Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, vol. 20, no. 2, 2009, pp. 89-100.
Batlle J, López-Fernández MF, Fraga EL, et al. Von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease. Blood Coagul Fibrinolysis. 2009;20(2):89-100.
Batlle, J., López-Fernández, M. F., Fraga, E. L., Trillo, A. R., & Pérez-Rodríguez, M. A. (2009). Von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease. Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, 20(2), pp. 89-100. doi:10.1097/MBC.0b013e3283254570.
Batlle J, et al. Von Willebrand Factor/factor VIII Concentrates in the Treatment of Von Willebrand Disease. Blood Coagul Fibrinolysis. 2009;20(2):89-100. PubMed PMID: 19786936.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease. AU - Batlle,Javier, AU - López-Fernández,María Fernanda, AU - Fraga,Esther Lourés, AU - Trillo,Angela Rodríguez, AU - Pérez-Rodríguez,María Almudena, PY - 2009/9/30/entrez PY - 2009/9/30/pubmed PY - 2009/12/16/medline SP - 89 EP - 100 JF - Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis JO - Blood Coagul. Fibrinolysis VL - 20 IS - 2 N2 - Therapy for von Willebrand disease (VWD) aims to restore the hemostatic function conferred by von Willebrand factor (VWF), which facilitates platelet adhesion and aggregation, and serves to increase potentially low coagulation factor VIII (FVIII) in plasma. In patients unresponsive to desmopressin (DDAVP), the preferred treatment is with plasma-derived VWF-containing FVIII concentrates. Only a few of the available VWF/FVIII concentrates have been licensed for use in VWD based on prospective studies. The efficacy of VWF/FVIII concentrates depends on the content and quality of VWF and FVIII. Several studies have demonstrated the variability of the VWF contents, as well as the differences in the VWF multimer patterns (including the high molecular weight VWF multimers that are most effective in restoring hemostasis), among these concentrates. Treating physicians should be aware of these disparities and the potential clinical implications for patients with different VWD subtypes. Dosing has traditionally been calculated based on the FVIII content of the products, although dosing based on VWF functional activity [e.g., VWF ristocetin cofactor activity (VWF:RCo)] addresses the primary protein deficiency in VWD patients. Several clinical studies have demonstrated the efficacy of concentrates dosed according to VWF:RCo. Dosing is generally consistent across VWD subtypes, although patients with severe phenotypes or undergoing major procedures may require more infusions or longer treatment duration. Other considerations for the use of VWF-containing concentrates include laboratory monitoring of efficacy and safety issues such as thrombosis risk and thromboprophylaxis. SN - 1473-5733 UR - https://www.unboundmedicine.com/medline/citation/19786936/Von_Willebrand_factor/factor_VIII_concentrates_in_the_treatment_of_von_Willebrand_disease_ L2 - http://dx.doi.org/10.1097/MBC.0b013e3283254570 DB - PRIME DP - Unbound Medicine ER -