Journal
EXPERT REVIEW OF HEMATOLOGY
Volume 7, Issue 5, Pages 573-581Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1586/17474086.2014.947955
Keywords
hemophilia B; inhibitors; long-acting FIX products; plasma-derived FIX concentrates; recombinant FIX products
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Hemophilia B is a rare hereditary hemorrhagic disorder characterized by deficiency of the clotting factor IX (FIX). Hemophilia B patients experience mild-to-severe bleeding complications according to the degree of FIX defect. The mainstay of treatment of hemophilia B consists of replacement therapy and nowadays several plasma-derived and recombinant FIX products are commercially available. This article reviews the current management of hemophilia B patients analyzing the results of the most important clinical trials. In addition, it will focus on the more recent advances in the production of new FIX molecules aimed at the improvement of the clinical management of such patients.
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