Preface

Not long ago, when La Paz Haemophilia Centre was created, we could not imagine the great advances we would see in respect to orthopaedic surgery for haemophilic patients. Since 1971, with a more systematic approach, we have also been able to provide curative as well as preventive surgery, such as knee synovectomies. We were working towards a goal of being able to stop the haemarthroses in order to halt the evolution of haemophilic arthropathy. Those patients treated with cryoprecipitates did not bleed, but the low purity of these products with a high proportion of proteins other than factor VIII, produced reactions to heterologous proteins, a low degree of haemolysis in cases of ABO incompatibility, or generation of immune complexes. The programmed surgery was slowed down during a number of years because of two reasons: the secondary reactions previously described, and overall, the limits of cryoprecipitate production. Cryoprecipitates were obtained from blood bags drawed for haemotherapy at the hospital, and most of them were used shortly afterwards in the treatment of acute bleedings. In case of a programmed surgical intervention it was necessary to store cryoprecipitates over a matter of months, to achieve enough to cover surgery, the postoperative period and the postoperative rehabilitation. This work was facilitated with the appearance of the ®rst antihaemophilic factor VIII and factor IX concentrates. It was in the early 1980s when the use of intensive substitutive therapy begun, trying to transform the haemophilic patient to a normal person. Those were the golden years for orthopaedic surgery. Orthopaedic surgeons were more and more audacious; the absence of haemorrhages, both peroperatively and postoperatively, let them to carry out every surgical technique being applied to normal people: osteotomies, arthroplasties, tendon lengthenings, and an intensive postoperative physiotherapy. Unfortunately AIDS emerged in 1983; although the infection had arisen in previous years; we are still living its terrible sequela of deaths and regrets. At those moments, we passed by a period of worldwide amazement. Surgery was stopped in many haemophilia centres because of di€erent reasons: concentrates were highly likely to be vehicles of infection; surgery could further worsen the deteriorated immunological situation of patients; and ®nally, the surgical team had a high risk of became infected through patients' blood during the surgical procedures. Every one of these problems was overcome. The arrival of puri®ed and virus-inactivated concentrates removed the initial fears. Moreover it was demonstrated that the infection risk for health sta€ was within tolerable levels, provided that adequate prevention measures were taken, and ®nally it was also demonstrated that the immunological deterioration was related to the patient's previous situation. In our centre it was decided to operate only on emergency cases, and among the programmed, if it was absolutely necessary, those that had no opportunistic infections and had more than 200 CD4 lymphocytes. These fears have been resolved through the years and their impediments have been removed step by step. The ultrapuri®cation of factor IX concentrates have practically surpassed all thromboembolic complications of prothrombin complex concentrates. However, in my opinion, it is better to maintain prophylactic low molecular heparin in those cases with high thrombogenic risk. Recently, the new factor VII activated recombinant concentrate seems to enable surgery among haemophiliacs with inhibitors, improving their quality of life, to whom very little could be o€ered until now. Current treatment for haemophiliacs, especially in countries with adequate economic resources, will lead in the coming years to a decrease in deformities in adults requiring surgical correction. This will be possible with the generalization of prophylactic treatment for all severe haemophiliacs, from their ®rst years of life or after the ®rst or second haemarthrosis in a particular joint. Prophylaxis has demonstrated its ecacy in preventing the development of the crippling conditions linked to haemophilia, because of the decrease in the number of haemorrhages. But currently 80% of haemophiliacs wordlwide do not receive any antihaemophilic treatment. Usually they live in countries with low economic levels: prophylaxis would Haemophilia (1999), 5, (Suppl. 1), 1±2