TY - CHAP M1 - Book, Section TI - Coagulation Disorders A1 - Trinkman, Heidi A1 - McCavit, Timothy L. A1 - Duran, Lauren A2 - DiPiro, Joseph T. A2 - Yee, Gary C. A2 - Posey, L. Michael A2 - Haines, Stuart T. A2 - Nolin, Thomas D. A2 - Ellingrod, Vicki PY - 2020 T2 - Pharmacotherapy: A Pathophysiologic Approach, 11e AB - KEY CONCEPTSHemophilia 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 their resulting long-term complications, and to arrest bleeding if it occurs.Recombinant factor concentrates usually are the first-line treatment of hemophilia because they have the lowest risk of infection.Inhibitor formation is the most significant treatment complication in hemophilia and 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 who have 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 vWD, and for serious bleeding in type 1 vWD.Desmopressin acetate often is effective for the treatment of type 1 vWD. It also may be effective for the 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 - 2024/04/19 UR - accesspharmacy.mhmedical.com/content.aspx?aid=1182456085 ER -