title: Replacement therapy with virus-inactivated plasma concentrates in von Willebrand disease.
abstract: In von Willebrand disease, the goal of treatment is to correct the two laboratory hallmarks of abnormal hemostasis, i.e. the deficiency of factor VIII (FVIII) and the prolonged bleeding time (BT). Since desmopressin (DDAVP) is able to achieve both these goals in the majority of patients, it is the treatment of choice. Some patients, however, are not responsive or become refractory to DDAVP. In these, blood products maintain an important therapeutic role, and there is a need to assess the efficacy of the recently available virus-inactivated plasma concentrates, which contain both FVIII and von Willebrand factor and carry a low risk of transmitting blood-borne viruses. Our survey of the data reported in the literature indicates that all available concentrates are similarly effective in attaining high and sustained levels of FVIII after infusion. Although they often shorten or normalize the prolonged BT, that effect is less uniform. Since concentrates appear efficacious in the majority of clinical situations that require the use of blood products, they should be preferred, because of their greater safety, to cryoprecipitate produced by blood banks, which cannot be virus inactivated.
authors.affiliation: Department of Hematology, San Bortolo Hospital, Vicenza, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Rodeghiero
authors.forename: G
authors.initials: G
authors.lastname: Castaman
authors.forename: D
authors.initials: D
authors.lastname: Meyer
authors.forename: P M
authors.initials: PM
authors.lastname: Mannucci
mesh_terms: D001760:Bleeding Time; D016913:Blood Component Transfusion / Q000592:standards; D003131:Combined Modality Therapy; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D003902:Detergents / Q000494:pharmacology; D005612:Freeze Drying; D006358:Hot Temperature; D006801:Humans; D010949:Plasma / Q000382:microbiology; D015995:Prevalence; D012449:Safety; D012997:Solvents / Q000494:pharmacology; D014777:Virus Diseases / Q000517:prevention & control / Q000635:transmission; D014780:Viruses / Q000187:drug effects; D014842:von Willebrand Diseases / Q000188:drug therapy / Q000453:epidemiology / Q000628:therapy*; D014841:von Willebrand Factor / Q000302:isolation & purification / Q000627:therapeutic use
chemical_list: D003902:Detergents; D012997:Solvents; D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin