New treatments of von Willebrand disease: plasma derived von Willebrand factor concentrates.
During the past 10 years the quality of plasma derived virus inactivated products containing von Willebrand factor (vWF) has improved and the ratios of ristocetin cofactor activity (vWF:RCo) to von Willebrand factor antigen (vWF:Ag) have increased. There are, however, considerable variations in AHF-vWF products with ranges from 0.25 to 1.4 unit of vWF:RCo for each unit of vWF:Ag, and 0.5 to 5.3 units of vWF:RCo for each factor VIIIC (FVIIIC) unit. The availability of a vWF product depleted of FVIII has allowed pharmacokinetic studies of the vWF protein. There have been no dose response studies, dosage regimens remain empirical and are still, except in rare instances, based on FVIIIC dosage. Current concentrates are as effective as cryoprecipitate in the management of patients with vWD non responsive to DDAVP. These concentrates should be preferred to cryoprecipitate which carries a risk of virus transmission.
Menache
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Biomedical Services, Amarican Red Cross, Washington, DC, USA.
Aronson
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D000941:Antigens / Q000097:blood; D002986:Clinical Trials as Topic; D006801:Humans; D012307:Risk Factors; D014842:von Willebrand Diseases / Q000097:blood / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000276:immunology / Q000378:metabolism / Q000627:therapeutic use*
D000941:Antigens; D014841:von Willebrand Factor