title: Clinical experience with human plasma-derived factor VIIa in patients with hemophilia A and high titer inhibitors.
abstract: FVIIa purified from human plasma and spontaneously activated during the purification procedure was given to 4 patients with hemophilia A and inhibitors against FVIII:C in association with joint bleeds. A dose of 9-20 micrograms/kg b.w. (700-1,000 U/kg b.w.) seemed to be hemostatically active in moderate to severe joint bleeds. Plasma levels of FVII of 5-7 U/ml were achieved and recoveries varied between 17 and 66%. The lower recovery rates of 17-39% were all found in 1 of the patients. No immediate side effects were seen. Neither were any signs of a systemic activation of the coagulation system observed. It is concluded that highly purified FVIIa may be useful in the treatment of hemophilia A patients with inhibitors against FVIII:C.
authors.affiliation: Novo Research Institute, Bagsvaerd, Denmark.
authors.forename: U
authors.initials: U
authors.lastname: Hedner
authors.forename: S
authors.initials: S
authors.lastname: Bjoern
authors.forename: S S
authors.initials: SS
authors.lastname: Bernvil
authors.forename: L
authors.initials: L
authors.lastname: Tengborn
authors.forename: L
authors.initials: L
authors.lastname: Stigendahl
mesh_terms: D000328:Adult; D001772:Blood Cell Count; D005167:Factor VII / Q000302:isolation & purification / Q000627:therapeutic use*; D005169:Factor VIII / Q000276:immunology; D005340:Fibrinogen / Q000378:metabolism; D006467:Hemophilia A / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011517:Prothrombin Time
chemical_list: D005167:Factor VII; D005169:Factor VIII; D005340:Fibrinogen