The safety of plasma-derived von Willebrand/factor VIII concentrates in the management of inherited von Willebrand disease.
Until the mid-80s, cryoprecipitate has been the mainstay of treatment of patients with von Willebrand disease who were unresponsive to desmopressin. The advent of virally-inactivated factor VIII (FVIII) concentrates containing von Willebrand factor (VWF), originally devoted to hemophiliacs, provided a better therapeutic approach to von Willebrand disease. These VWF/FVIII concentrates were introduced in clinical practice after the positive results obtained in several prospective and retrospective clinical studies. They are safe and can be suitable also for home treatment. Allergic or anaphylactic reactions are limited to the rare patients with deletions of VWF gene. In repeated infusions during surgery, the dosage and timing of administration should be planned to keep FVIII below 150 - 200 U/dl to avoid any possible risk of thrombosis.
Federici
Augusto B
AB
Department of Medicine and Medical Specialties, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Milano, Italy. augusto.federici@unimi.it
D003029:Coagulants / Q000009:adverse effects / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004338:Drug Combinations; D005169:Factor VIII / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D016896:Treatment Outcome; D038301:Virus Inactivation; D014842:von Willebrand Diseases / Q000235:genetics / Q000628:therapy; D014841:von Willebrand Factor / Q000009:adverse effects / Q000627:therapeutic use
D003029:Coagulants; D004338:Drug Combinations; D014841:von Willebrand Factor; D005169:Factor VIII