Joint replacement for the management of haemophilic arthropathy in patients with inhibitors: A long-term experience at a single Haemophilia centre

Introduction: The association between haemophilia and the so-called 'inhibitors', alloantibodies against the infused factor able to neutralize its clotting activity, is a very rare condition. Those sporadic patients suffer of an even more severe arthropathy and performing primary or revision arthroplasty become truly challenging. Literature about this topic is scarce, consisting in small case series, high rates of complications and mid-term follow-ups. Aim: The purpose of this study is the assessment of the long-term outcomes of primary and revision arthroplasty performed in a population of patients with inhibitors, the more consistent to date reported at a single haemophilia centre. Methods: We reviewed the records of 18 patients with inhibitors (26 procedures) between 1999 and 2017, divided in two groups. Group A [primary total Knee-Hip arthroplasty (TKA-THA)]: 13 patients underwent 19TKA and 2THA; and B (revision): 5 subjects underwent 3rTKA and 2rTHA. All patients received the same haematological prophylaxis (rFVIIa). Haemophilic Joint Health score and VAS, and X-rays were recorded pre- and postoperatively. The survival rate of all primary implants was assessed. Results: The median follow-up was 12.2 years (3-21) for group A, 8.6 years (4-12) for B. Few complications have been reported; the overall survival rate was 94.7% at 15 years. All patients reported satisfaction, pain reduction and improved functional ability. Conclusion: Primary and revision TKA/THA in haemophilic subjects and inhibitors may be nowadays considered safe and effective if performed in dedicated multidisciplinary centres. The use of continuous infusion of rFVIIa showed an adequate haemostatic effect and low rate of complications. As expected, revisions are more prone to complications compared to primary arthroplasty.