title: Use of recombinant factor VIIa in 3 patients with inherited type I Glanzmann's thrombasthenia undergoing invasive procedures.
abstract: The treatment of bleeds in Glanzmann's thrombasthenia is a challenging issue, especially when repeated platelet transfusions have induced anti-glycoprotein (GP) IIb-IIIa or anti-HLA allo-immunisation. In an attempt to find an alternative treatment regimen, we used recombinant factor VIIa (rFVIIa, NovoSeven, Novo Nordisk, Denmark) as first-line therapy in 3 patients with Glanzmann's thrombasthenia and anti-GPIIb-IIIa iso-antibodies who were scheduled for invasive procedures. The administration of an initial bolus dose of rFVIIa (70-110 microg/kg) was immediately followed by continuous infusion at the rate of 9-30 microg/kg/h for 3-15 days. The treatment resulted in an excellent clinical efficacy and tolerance in 2 cases. In the third patient, whereas efficacy was excellent at the surgical site, pharyngonasal bleeds of traumatic origin persisted for 10 days, and a severe thromboembolic complication occurred 5 days after discontinuation of rFVIIa. Complementary studies are needed for patients with congenital platelet disorders in order to evaluate the safety and the potential therapeutic place of rFVIIa treatment.
authors.affiliation: The Haemophilia Center, Hôpital Bicêtre, Paris Sud, France. rdoiron@calva.net
authors.forename: R
authors.initials: R
authors.lastname: d'Oiron
authors.forename: C
authors.initials: C
authors.lastname: Ménart
authors.forename: M C
authors.initials: MC
authors.lastname: Trzeciak
authors.forename: P
authors.initials: P
authors.lastname: Nurden
authors.forename: E
authors.initials: E
authors.lastname: Fressinaud
authors.forename: M
authors.initials: M
authors.lastname: Dreyfus
authors.forename: Y
authors.initials: Y
authors.lastname: Laurian
authors.forename: C
authors.initials: C
authors.lastname: Négrier
mesh_terms: D000230:Adenocarcinoma / Q000150:complications / Q000601:surgery; D000328:Adult; D000368:Aged; D002763:Cholecystectomy; D003082:Colectomy; D003110:Colonic Neoplasms / Q000150:complications / Q000601:surgery; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D007813:Laparotomy; D008297:Male; D008875:Middle Aged; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000276:immunology; D011994:Recombinant Proteins / Q000627:therapeutic use; D013915:Thrombasthenia / Q000150:complications / Q000188:drug therapy*
chemical_list: D019039:Platelet Glycoprotein GPIIb-IIIa Complex; D011994:Recombinant Proteins; D015942:Factor VIIa