title: Ten-year experience of recombinant activated factor VII use in surgical patients with congenital haemophilia with inhibitors or acquired haemophilia in Japan.
abstract: Patients with congenital haemophilia with inhibitors or acquired haemophilia are at risk of bleeding complications during surgery. In these patients, replacement therapy for the missing coagulation factor is ineffective, and a bypassing agent such as recombinant activated factor VII (rFVIIa) is required to manage bleeding. To evaluate the safety and haemostatic efficacy of rFVIIa treatment in Japanese patients with congenital haemophilia with inhibitors to FVIII/FIX or acquired haemophilia undergoing surgery. Postmarketing surveillance data from May 2000 to March 2010 were analysed to assess the haemostatic efficacy of 38 procedures in 22 patients with congenital haemophilia A, 13 procedures in seven patients with congenital haemophilia B, and five procedures in five patients with acquired haemophilia. Postoperative bleeding control was judged to be effective (bleeding was stopped completely or reduced considerably) for 34/38 procedures (89%) in patients with congenital haemophilia A, 10/13 procedures (77%) in patients with congenital haemophilia B, and 4/5 procedures (80%) in patients with acquired haemophilia. Tranexamic acid was used concomitantly for 36/56 procedures (64%). Safety was analysed for 66 procedures in 37 patients. Adverse effects potentially related to rFVIIa treatment included mild superficial thrombophlebitis, mild decrease in platelet count, and mild elevation of the serum alanine transaminase level in one patient each. All adverse effects resolved without treatment. Administration of rFVIIa provided adequate haemostasis without serious adverse effects in the majority of cases. The efficacy and safety data in Japanese patients were similar to previously published data from other countries.
authors.affiliation: Hospital of the Institute of Medical Science of the University of Tokyo (IMSUT), Tokyo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Takedani
authors.affiliation: Nara Medical University, Kashihara, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Shima
authors.affiliation: Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Horikoshi
authors.affiliation: Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Koyama
authors.affiliation: Tokyo Medical University, Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Fukutake
authors.affiliation: Novo Nordisk Pharma Ltd., Tokyo, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kuwahara
authors.affiliation: Nagoya University School of Medicine, Nagoya, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Ishiguro
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D005169:Factor VIII / Q000276:immunology; D015942:Factor VIIa / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology* / Q000601:surgery; D002836:Hemophilia B / Q000188:drug therapy* / Q000276:immunology* / Q000601:surgery; D006801:Humans; D007223:Infant; D007518:Isoantibodies / Q000097:blood / Q000276:immunology; D007564:Japan; D008297:Male; D008875:Middle Aged; D019106:Postoperative Hemorrhage / Q000517:prevention & control*; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D007518:Isoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa
keywords: Japan; continuous infusion; haemophilia with inhibitors; postmarketing surveillance; recombinant factor VIIa; surgery