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.

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

“…Contemporary surgical and haematological advances (FEIBA, rFVIIa) allow us to perform major operations on haemophilic patients with inhibitors, however, also with a higher risk of bleeding complications than in haemophilic patients without inhibitors [14][15][16].…”

A Jehovah’s Witness child with hemophilia B and factor IX inhibitors undergoing scoliosis surgery

“…3 Annual inhibitor screening should be done as part of the comprehensive care for hemophilia patients. Screening was not done prior to the anaphylactic episode in our patient as he was unable to attend the hemophilia clinic.…”

“…2 This represents the greatest complication in replacement therapy and has rendered the treatment of hemophilia less straightforward. 3 Anaphylaxis develops contemporaneously with the appearance of inhibitor in half of these patients. 2 Although the frequency of spontaneous bleeding is not necessarily increased in patients with inhibitors, the level of the inhibitor and the magnitude of the individual's anamnestic response to factor challenge dictate the outcome of hemostasis.…”

“…2 Although the frequency of spontaneous bleeding is not necessarily increased in patients with inhibitors, the level of the inhibitor and the magnitude of the individual's anamnestic response to factor challenge dictate the outcome of hemostasis. 3 Good control of hemostasis is particularly important in patients with inhibitors who are scheduled for surgery.…”

Sequential bypassing agents during major orthopedic surgery: a new approach to hemostasis

“…5 But over the past decade, with the use of bypassing agents (BPAs), recombinant activated factor VII (rFVIIa), and activated prothrombin complex concentrate (APCC), surgery has become more commonplace. 6 Yet orthopedic surgery, often necessary because of advanced arthropathy from repeated hemarthrosis, is frequently avoided in inhibitor patients, despite a 2.5-fold decrease in joint mobility and a negative association between health-related quality of life and orthopedic condition. 7,8 Nevertheless, several published reports have demonstrated the feasibility of orthopedic surgery with BPAs in this cohort, and consensus recommendations have been developed detailing their use.…”