Preoperative planning should incorporate a determination of the specific haemostatic therapies to be used during and after surgery, including dosing regimens and whether continuous selleck or bolus treatment will be used. It is often

helpful for a member of the HTC team to be present in the operating room (OR) to assist in communication between the OR staff and the patient/family and to provide on-site guidance regarding haemostatic management, if needed. The use of high-dose FVIII or FIX concentrates to overcome inhibitors in CHwI undergoing surgery, although ideal and measurable [8, 21], is often restricted to those with low-titre or low-responding inhibitors or those who have successfully achieved tolerance. Both bolus and continuous administration of replacement factor have been 3-deazaneplanocin A effectively used in this setting, although in patients with haemophilia B and inhibitors, the use of high doses of FIX may increase the risk for anaphylaxis [10]. In patients with

haemophilia A receiving FVIII replacement for surgery, an anamnestic increase in inhibitor titre may occur, necessitating a switch to bypassing therapy [22]. Although preoperative attempts to reduce the inhibitor titre using rituximab [9] and ITT [6] have been described, these treatments have limitations, most notably the time required for such regimens to take effect and, with immunosuppressive eliminative agents, the potential for susceptibility to infections. Bypassing agents are the haemostatic products of choice for patients with high-titre or high-responding inhibitors or those with haemophilia B and inhibitors. Each of the commercially available Exoribonuclease bypassing agents – recombinant activated FVII (rFVIIa; NovoSeven® RT; Novo Nordisk A/S, Bagsvaerd, Denmark) and activated

prothrombin complex concentrate (aPCC; FEIBA®, Factor Eight Inhibitor Bypassing Agent); Baxter Healthcare Corporation (Westlake Village, CA, USA) – have been successfully used for haemostatic coverage for surgery in both children and adults with CHwI, with comparable efficacy and safety. However, there are no evidence-based guidelines for the use of either agent in this setting. Recombinant FVIIa has a relatively short half-life of 2.7 h in adults and 1.3 h in children [23]. Optimal dosing remains uncertain. The choice of product for those with high-titre inhibitors is dependent on the age of the patient, prior exposure to plasma products, type of bleeding episode, volume-of-reconstitution cost, efficacy and safety. At most institutions, for patients who are plasma-naïve or for those with haemophilia B and inhibitors, rFVIIa is used to achieve rapid haemostasis (recombinant porcine FVIII may likewise be used for the same purpose in patients with haemophilia A and inhibitors who are plasma-naïve, when it becomes available). However, for patients with haemophilia A who have been previously exposed to plasma products, either aPCC or rFVIIa may be used [24].