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.

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Total knee arthroplasty (TKA) is associated with blood loss, requiring blood transfusion in 44% of patients on average (range of 9-84%) [1]. Blood transfusion remains a standard treatment in case of postoperative anaemia after TKA, and in many institutions two packed red blood cell units are routinely prepared preoperatively for TKAs. However, the risks of blood transfusions are well established [2]. Many authors have reported the efficacy and safety of intravenous [3-7] and intra-articular [8,9] tranexamic acid (TXA) in preventing blood loss during TKA.The use of a multimodal blood loss prevention approach (MBLPA) that includes intra-articular TXA (MBLPA-TXA) has been shown to diminish the transfusion rate in TKA for persons without haemophilia [7][8][9]. However, the transfusion rates, haematological parameters that assess the prevention of blood loss, factor consumption and cost of surgery have not yet been defined in persons with haemophilia (PWH) when using the MBLPA-TXA. Our aim is to demonstrate that the MBLPA-TXA decreases the rate of postoperative blood transfusions by preventing blood loss, diminishing factor consumption in PWH.We conducted a retrospective case-control comparative study, including 10 PWH [severe haemophilia Aless than 1% factor VIII (FVIII) basal activity] with unilateral primary TKA at our institution. We included all PWH undergoing primary TKA in our institution since 1 January 2013 to 31 December 2013 (we currently perform around 10 TKAs per year in our centre). Patients were not selected to show a positive effect. The same surgeons did all the procedures and all patients were nursed in the same hospital on the same ward. Sex and preoperative haemoglobin (Hb) and haematocrit (Hct) were the same in both groups. The surgical technique was identical in both groups.

Since allogeneic blood transfusion (ABT) is not harmless, multiple alternatives to ABT (AABT) have emerged, though there is great variability in their indications and appropriate use. This variability results from the interaction of a number of factors, including the specialty of the physician, knowledge and preferences, the degree of anemia, transfusion policy, and AABT availability. Since AABTs are not harmless and may not meet cost-effectiveness criteria, such variability is unacceptable. The Spanish Societies of Anesthesiology (SEDAR), Hematology and Hemotherapy (SEHH), Hospital Pharmacy (SEFH), Critical Care Medicine (SEMICYUC), Thrombosis and Hemostasis (SETH) and Blood Transfusion (SETS) have developed a Consensus Document for the proper use of AABTs. A panel of experts convened by these 6 Societies have conducted a systematic review of the medical literature and have developed the 2013 Seville Consensus Document on Alternatives to Allogeneic Blood Transfusion, which only considers those AABT aimed at decreasing the transfusion of packed red cells. AABTs are defined as any pharmacological or non-pharmacological measure aimed at decreasing the transfusion of red blood cell concentrates, while preserving patient safety. For each AABT, the main question formulated, positively or negatively, is: « Does this particular AABT reduce the transfusion rate or not?» All the recommendations on the use of AABTs were formulated according to the Grades of Recommendation Assessment, Development and Evaluation (GRADE) methodology.

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