TY - CHAP M1 - Book, Section TI - Coagulation Disorders A1 - Trinkman, Heidi A1 - Beam, Donald A1 - Hagemann, Tracy A2 - DiPiro, Joseph T. A2 - Talbert, Robert L. A2 - Yee, Gary C. A2 - Matzke, Gary R. A2 - Wells, Barbara G. A2 - Posey, L. Michael PY - 2017 T2 - Pharmacotherapy: A Pathophysiologic Approach, 10e AB - KEY CONCEPTS Hemophilia is an inherited bleeding disorder resulting from a congenital deficiency in factor VIII or IX. The goal of therapy for hemophilia is to prevent bleeding episodes, and as a result their long-term complications, and to arrest bleeding if it occurs. Recombinant factor concentrates usually are first-line treatment of hemophilia as they have the lowest risk of infection. Inhibitor formation is the most significant treatment complication in hemophilia. It is associated with significant morbidity and decreased quality of life. Recombinant factor VIIa is effective for the treatment of acute bleeds in patients with hemophilia A or B that has developed inhibitors. The goal of therapy for von Willebrand disease (vWD) is to increase von Willebrand factor (vWF) and factor VIII levels to prevent bleeding during surgery or arrest bleeding when it occurs. Factor VIII concentrates that contain vWF are the agents of choice for treatment of type 3 vWD and some type 2 von Willebrand disease, and for serious bleeding in type 1 von Willebrand disease. Desmopressin acetate often is effective for treatment of type 1 vWD. It also may be effective for treatment of some forms of type 2 vWD in addition to mild to moderate hemophilia A. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesspharmacy.mhmedical.com/content.aspx?aid=1145204400 ER -