title: A standardized treatment regimen for patients with severe haemophilia A undergoing orthopaedic or trauma surgery: a single centre experience.
abstract: Recommendations on the administration of clotting factor concentrates in patients with severe haemophilia undergoing surgery are usually determined by monitoring target clotting factor levels. In this retrospective cohort study, we enrolled patients with severe haemophilia A who underwent major orthopaedic or trauma surgery. We wanted to evaluate the feasibility and the safety of a standardized medical treatment procedure. Further on, we wanted to assess whether our standardized treatment regimen enables surgical procedures in certain situations in which measuring clotting factor VIII (FVIII) activity is not available. We created a standardized medical treatment procedure that included a medical protocol and close cooperation with the Haemophilic Treatment Centre. Thirteen surgical procedures in nine patients were examined. The feasibility and safety of this standardized treatment concept were assessed by identifying perioperative complications and by means of a questionnaire. Depending on the surgery, the amount of FVIII administered within the first 10 days ranged between 653 and 1027 units/kg body weight. No allogeneic blood transfusion was required. The measurement of FVIII activity was performed repeatedly in five patients. In all patients activated partial thromboplastin time monitoring was performed during the hospital stays. The surgeons and patients were satisfied with our treatment concept and adhered to the medical regimen protocol. By means of a detailed, standardized medical protocol and by ensuring close cooperation between the patient, the surgeons and the Haemophilic Treatment Centre, we could show that elective and emergency operations can be safely performed even in situations in which FVIII activity could not be monitored.
authors.affiliation: aDepartment of Internal Medicine III, University of Regensburg, Regensburg bDepartment of Anaesthesiology, University of Munich, Munich cDepartment of Transfusion Medicine and Haemostasis, Klinikum Augsburg, Germany.
authors.forename: Christina
authors.initials: C
authors.lastname: Hart
authors.forename: Bernhard
authors.initials: B
authors.lastname: Heindl
authors.forename: Michael
authors.initials: M
authors.lastname: Spannagl
authors.forename: Susanne
authors.initials: S
authors.lastname: Lison
mesh_terms: D000328:Adult; D001803:Blood Transfusion; D005169:Factor VIII / Q000008:administration & dosage / Q000627:therapeutic use*; D006467:Hemophilia A / Q000097:blood / Q000150:complications* / Q000628:therapy*; D006801:Humans; D008875:Middle Aged; D009985:Orthopedics; D012189:Retrospective Studies; D014947:Wounds and Injuries / Q000097:blood / Q000150:complications* / Q000601:surgery*
chemical_list: D005169:Factor VIII