Inherited
factor VII (FVII) deficiency is a rare coagulation disorder with variable haemorrhagic manifestations. In severely affected cases spontaneous haemarthroses leading to advanced
arthropathy have been observed. Such cases may require surgery. Therapeutic options for
bleeding prevention in FVII deficient patients undergoing surgery comprise various FVII preparations but the use of recombinant
activated factor VII (
rFVIIa) seems to be the treatment of choice. To present the outcome of orthopaedic surgery under haemostatic coverage of
rFVIIa administered according to the locally established treatment regimen in five adult patients with FVII baseline plasma levels below 10 IU dL(-1). Two patients required
total hip replacement (THR); three had various arthroscopic procedures. Recombinant
activated factor VII was administered every 8 h on day of surgery (D0) followed by every 12-24 h for the subsequent 9-14 days, depending on the type of surgery.
Factor VII plasma coagulation activity (
FVII:C) was determined daily with no predefined therapeutic target levels. Doses of
rFVIIa on D0 ranged from 18 to 37 μg kg(-1) b.w. and on the subsequent days--from 13 to 30 μg kg(-1) b.w. Total
rFVIIa dose per procedure ranged from 16 to 37.5 mg, and the total number of doses per procedure was 16-31. None of our patients developed excessive
bleeding including those in whom
FVII:C trough levels returned nearly to the baseline level on the first post-op day. Preliminary results demonstrate that
rFVIIa administered according to our treatment regimen is an effective and safe haemostatic agent for hypoproconvertinaemia patients undergoing orthopaedic surgery.