title: Recombinant factor VIIa is effective for bleeding and surgery in patients with Glanzmann thrombasthenia.
abstract: Recombinant activated factor VII (rFVIIa) was found to be effective and safe in treating 24 bleeding episodes and to prevent bleeding during one bilateral herniorrhaphy in four children with Glanzmann thrombasthenia. One of the patients had alloantibodies to platelet membrane glycoprotein (GP) IIb/IIIa and was refractory to platelet transfusion. rFVIIa was administered at 89 to 116 microg/kg per injection every 2 hours, in association with antifibrinolytic drugs. Bleeding stopped in all cases, but platelet transfusion was required in one. Two bleeding episodes recurred 36 and 63 hours after discontinuation of rFVIIa, but were successfully treated with additional doses. No adverse effects of rFVIIa were observed. Although the number of patients is small, our study suggests that rFVIIa may be an alternative to platelet transfusions in patients with a severe congenital thrombocytopathy.
authors.affiliation: Departments of Medicine, Pediatrics, and Oncology, University of Calgary and Foothills Hospital, Calgary, Alberta, Canada. mcpoon@ucalgary.ca
authors.forename: M C
authors.initials: MC
authors.lastname: Poon
authors.forename: C
authors.initials: C
authors.lastname: Demers
authors.forename: F
authors.initials: F
authors.lastname: Jobin
authors.forename: J W
authors.initials: JW
authors.lastname: Wu
mesh_terms: D016063:Blood Loss, Surgical / Q000517:prevention & control*; D002648:Child; D002675:Child, Preschool; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006470:Hemorrhage / Q000188:drug therapy*; D006801:Humans; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use*; D013915:Thrombasthenia / Q000188:drug therapy* / Q000503:physiopathology / Q000601:surgery; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D015942:Factor VIIa