Studies of Factor VIII Inhibitors in Chinese Hemophilia a Patients
It has been recognized that hemophilic patients with clotting factor inhibitors bleed no more frequently and no worse than patients who do not have this complication. However, the management of bleeding in these patients is a major problem. Low responders can be treated effectively with high doses of clotting factor, whereas treatment is difficult when the clotting factor inhibitor titer is high. Therefore, the diagnosis of the presence of clotting factor inhibitor, immunologic characterization of the inhibitor and investigation of the interaction of the inhibitor with clotting factor become very important in the management of the hemophiliacs with inhibitor. Recent advances in the development of gene cloning and expression have made it possible to characterize in detail, the epitopes recognized by inhibitors. This epitope mapping helps us to understand the structure and function relationship of factor VIII protein and also the mechanism by which these factor VIII inhibitors react[2,3]. This paper is to present the immunologic characterization and kinetic studies of factor VIII inhibitors found in six patients and epitope mapping of one of the inhibitors.
KeywordsFactor VIII Thrombin Activation Hemophilic Patient Translate Amino Acid Sequence Immunologic Characterization
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