Surgery in haemophilia patients with inhibitors, with special emphasis on orthopaedics: Madrid experience.
We report on a series of 92 surgical procedures (90 patients). It includes 35 orthopaedic procedures (33 patients) and 57 non-orthopaedic procedures (57 patients). The orthopaedic procedures include 27 radiosynovectomies (minor surgery) and eight major orthopaedic procedures. The non-orthopaedic procedures include 52 minor interventions and five major procedures. The average age of patients was 34 years (range: 8-56), and the average follow-up time was 3 years (range: 1-6). Of the 92 surgical procedures, 42 were performed with activated prothrombin complex concentrates [factor eight inhibitor bypassing agent (FEIBA)] and 47 with recombinant-activated factor VIIa (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark). Regarding FEIBA treatment in minor surgery, the initial dose was 100 IU kg(-1). After 6 h, we continued with 50 IU kg(-1) every 12 h for at least 4 days (radiosynovectomies). In minor non-orthopaedic procedures, the dose was continued until day 14. In patients who underwent surgery with the haemostatic control achieved by means of rFVIIa, the initial dose of rFVIIa in minor procedures (both orthopaedic and non-orthopaedic) was 90-120 microg kg(-1). In postoperative days 1-5, the dose was 2-4 x 90-120 microg kg(-1) q3-6 h for 24 h. In major procedures (both orthopaedic and non-orthopaedic), the dose was 120 microg kg(-1) pre-operatively, 120 microg kg(-1) q 3 h day 2/day 3-5, and then 90-120 microg kg(-1) q 6 h until day 14. There were 87 good results, four fair results and one poor result. Our study has shown that haemophilic patients with inhibitors requiring surgery can undergo orthopaedic and non-orthopaedic procedures with a high expectation of success. In other words, surgery (orthopaedic and non-orthopaedic) is now possible in haemophilia patients with inhibitors, leading to an improved quality of life for these patients.
Department of Orthopaedics and Haemophilia Unit, La Paz University Hospital, Madrid, Spain. carlosrodriguezmerchan@gmail.com
E C
EC
Rodriguez-Merchan
V
V
Jimenez-Yuste
P
P
Gomez-Cardero
M
M
Alvarez-Roman
M
M
Martin-Salces
A
A
Rodriguez de la Rua
D000293:Adolescent; D000328:Adult; D001779:Blood Coagulation Factors / Q000008:administration & dosage* / Q000627:therapeutic use; D016063:Blood Loss, Surgical / Q000517:prevention & control; D002648:Child; D004334:Drug Administration Schedule; D015942:Factor VIIa / Q000008:administration & dosage* / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D002836:Hemophilia B / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007592:Joint Diseases / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D019990:Perioperative Care; D011182:Postoperative Care; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D016896:Treatment Outcome; D055815:Young Adult
D001779:Blood Coagulation Factors; D011994:Recombinant Proteins; C103587:recombinant FVIIa; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa