title: US experience with recombinant factor VIIa for surgery and other invasive procedures in acquired haemophilia: analysis from the Hemostasis and Thrombosis Research Society Registry.
abstract: BACKGROUND
  Acquired haemophilia (AH) is a rare disorder caused by autoantibodies against factor VIII.
  
  
  OBJECTIVE
  The Hemostasis & Thrombosis Research Society (HTRS) Registry was used to monitor the safety of recombinant FVII (rFVIIa). This study aims to report data from the HTRS Registry regarding safety and efficacy of rFVIIa for haemostatic management of surgeries and other invasive procedures in patients with AH.
  
  
  METHODS
  For each rFVIIa-treated procedure, the initial dose, total dose, average infused dose, number of doses and treatment duration were calculated. Efficacy was assessed on a 4-point scale.
  
  
  RESULTS
  Of 166 registered patients with AH, 37 patients underwent 58 procedures [30 (51%) rFVIIa-treated]. The median (range) age of all patients undergoing procedures was 70 (13-93) years; for rFVIIa-treated patients, 74 (28-89) years. Approximately 67% (39/58) of all procedures were elective. Overall, the most common procedures were endoscopy (12) and central venous access device (10); rFVIIa was used preoperatively (11), postoperatively (13) and during six follow-up procedures during ongoing postoperative rFVIIa treatment. The median (range) initial dose was 90.0 (44-187) μg kg(-1) preoperatively and 106.0 (56-270) μg kg(-1) postoperatively. For rFVIIa-treated episodes with a reported outcome, 20 (91%) were rated excellent/good or no additional agents used and 2 (9%) were rated as poor/ineffective requiring a switch to another bypassing agent. No thromboembolic events were reported.
  
  
  CONCLUSIONS
  Adequate haemostasis was provided for 91% of rFVIIa-treated procedures at doses largely conforming to the package insert. No safety concerns were reported.
authors.affiliation: Division of Hematology/Oncology, University of North Carolina School of Medicine, Chapel Hill, NC, USA.
authors.forename: A D
authors.initials: AD
authors.lastname: Ma
authors.affiliation: Georgetown Lombardi Comprehensive Cancer Center, Georgetown University Medical Center, Washington, DC, USA.
authors.forename: C M
authors.initials: CM
authors.lastname: Kessler
authors.affiliation: Division of Hematology-Oncology, Penn State Milton S. Hershey Medical Center and Penn State College of Medicine, Hershey, PA, USA.
authors.forename: H A B
authors.initials: HA
authors.lastname: Al-Mondhiry
authors.affiliation: Clinical, Medical, and Regulatory Affairs, Novo Nordisk Inc., Plainsboro, NJ, USA.
authors.forename: R Z
authors.initials: RZ
authors.lastname: Gut
authors.affiliation: Clinical, Medical, and Regulatory Affairs, Novo Nordisk Inc., Plainsboro, NJ, USA.
authors.forename: D L
authors.initials: DL
authors.lastname: Cooper
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016208:Databases, Factual; D015942:Factor VIIa / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy* / Q000453:epidemiology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000009:adverse effects / Q000627:therapeutic use; D012042:Registries; D012449:Safety; D014481:United States; D055815:Young Adult
chemical_list: D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa
keywords: acquired haemophilia; haemostasis; rFVIIa; registry; safety; surgery