title: Surgery in haemophilic patients with inhibitor: 20 years of experience.
abstract: Surgery in haemophilic patients with inhibitor against factor (F)VIII or FIX is high risk. Surgery may be performed with the administration of sufficiently high dose of FVIII in patients with low-response inhibitor or who, despite having a high response, present a low inhibitor titre at the time of surgery. The use of high doses of FX is more complicated in patients with a low-titre FIX inhibitor, as there is a high risk of anaphylactic reactions. In the case of patients with high-titre inhibitors, several treatments have been proposed, such as porcine FVIII, recombinant FVIIa (rFVIIa), and activated prothrombin complex concentrate (APCC). We present our 20 years' experience in the treatment and subsequent management of haemophilic patients with inhibitor in surgery and evaluate the results obtained with the products available for haemostatic control in 64 surgical procedures. The efficacy we obtained with FVIII is good in 100% of the cases described; we had no haemorrhagic complication (HC) in the 18 procedures in which it was used (three major and 15 minor surgery). With APCC we obtained excellent results with only one HC in a synoviorthesis in the form of bleeding and haematomas out of 32 procedures. Good results were obtained with rFVIIa with few haemorrhagic episodes. Thus, in major surgery there was one HC out of three cases. In minor surgery, greater efficacy was observed using extremely large doses of rFVIIa (> or =120 mg kg(-1) 2 h(-1)) because of the shorter half-life of this factor in this type of patients.
authors.affiliation: Coagulopathies Centre, Department of Haematology, La Paz University Hospital, Madrid, Spain. mquintana.hulp@salud.madrid.org
authors.forename: M
authors.initials: M
authors.lastname: Quintana-Molina
authors.forename: F
authors.initials: F
authors.lastname: Martínez-Bahamonde
authors.forename: E
authors.initials: E
authors.lastname: González-García
authors.forename: J
authors.initials: J
authors.lastname: Romero-Garrido
authors.forename: A
authors.initials: A
authors.lastname: Villar-Camacho
authors.forename: V
authors.initials: V
authors.lastname: Jiménez-Yuste
authors.forename: I
authors.initials: I
authors.lastname: Fernández-Bello
authors.forename: F
authors.initials: F
authors.lastname: Hernández-Navarro
mesh_terms: D000293:Adolescent; D000328:Adult; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002405:Catheterization, Central Venous / Q000379:methods; D002648:Child; D002675:Child, Preschool; D005164:Factor IX / Q000037:antagonists & inhibitors; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D006467:Hemophilia A / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery*; D002836:Hemophilia B / Q000503:physiopathology / Q000601:surgery; D006487:Hemostasis / Q000502:physiology; D006801:Humans; D007223:Infant; D008875:Middle Aged; D019106:Postoperative Hemorrhage / Q000517:prevention & control; D011994:Recombinant Proteins / Q000627:therapeutic use; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D001779:Blood Coagulation Factors; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; D005169:Factor VIII; D005164:Factor IX; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa