Surgery in haemophilia patients with inhibitors, with special emphasis on orthopaedics: Madrid experience

Abstract

Summary. 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 nonorthopaedic) was 90-120 lg kg )1. In postoperative days 1-5, the dose was 2-4 · 90-120 lg kg )1 q3-6 h for 24 h. In major procedures (both orthopaedic and non-orthopaedic), the dose was 120 lg kg )1 preoperatively, 120 lg kg )1 q 3 h day 2/day 3-5, and then 90-120 lg 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.

Surgery in Inhibitor Patients

“…Oslo University Hospital, Oslo, Norway procedures performed using aPCC as replacement therapy in patients with inhibitors [2][3][4][5][6][7][8][9][10]. The hemostatic efficacy in these case series have been reported from 78% to 100%.…”

“…However, experience during the last 10-15 years using bypassing agents has shown that, despite increased bleeding risk compared to noninhibitor patients, the results are in general good [1][2][3][4][5][6][7][8][9][10][11][12]. However, experience during the last 10-15 years using bypassing agents has shown that, despite increased bleeding risk compared to noninhibitor patients, the results are in general good [1][2][3][4][5][6][7][8][9][10][11][12].…”

Recommendations on multidisciplinary management of elective surgery in people with haemophilia

“…Close collaboration and communication among the MDT members and the patient/family is vital throughout . Surgery in inhibitor patients remains the most challenging area and should only be conducted at an HTC by an experienced, specialized MDT …”

Comprehensive care of the patient with haemophilia and inhibitors undergoing surgery: practical aspects

“…However, for those with high‐titre inhibitors (≥5 BU), bypassing agents that circumvent the need for factor VIII (FVIII) or FIX concentrates are used to achieve haemostasis. Until recently, perioperative prophylaxis with bypassing agents was not considered in congenital haemophilia with inhibitors (CHwl) , and elective (especially major) surgery was rarely performed . Consequently, potentially beneficial surgeries and invasive screening procedures may have been deferred in this population, to the detriment of affected patients .…”