Modern aspects of hemophilia A diagnosis

Abstract

   The evolution of hemophilia treatment is rapidly developing. Both new factor replacement and non-factor therapy have appeared in recent years. One of the most important problems of factor replacement therapy is the relatively short half-life of coagulation factor VIII (FVIII), with an average of about 8–12 hours in adults, ranging in individual patients between 6 and 24 hours, and even shorter in younger children. This forces patients, especially children, to administer the drug quite often (3–4 times a week), reducing the quality of life and adherence to treatment. The appearance of recombinant FVIII products with an increased half-life allows to reduce the number of infusions per week, improving the quality of life of patients without compromising the safety and efficacy of treatment. However, the structure of these products leads to the changes in the results of laboratory tests of FVIII activity carried out to monitor the efficacy of treatment. In this article, we will consider the current methods of laboratory control of products with an increased half-life of FVIII currently available in Russia. We want to assess the discrepancy between the one-stage clotting method and chromogenic method for each FVIII product, as well as the laboratory's capabilities in monitoring non-factor and combined therapy for hemophilia A.