TY - CHAP M1 - Book, Section TI - Antibody-Mediated Coagulation Factor Deficiencies A1 - Stowell, Sean R. A1 - Lollar, Pete A2 - Kaushansky, Kenneth A2 - Prchal, Josef T. A2 - Burns, Linda J. A2 - Lichtman, Marshall A. A2 - Levi, Marcel A2 - Linch, David C. PY - 2021 T2 - Williams Hematology, 10e AB - SUMMARYClinically significant autoantibodies to coagulation factor deficiencies are uncommon but can produce life-threatening bleeding episodes and death. The most commonly targeted coagulation factor in autoimmunity is factor VIII. Acquired hemophilia A, which results from these antibodies, can be either idiopathic or associated with older age, other autoimmune disorders, malignancy, the postpartum period, or a drug reaction. Treatment options to prevent or stop bleeding in acquired hemophilia A include factor VIII–bypassing agents, anti-inhibitor coagulant complex and recombinant factor VIIa, and recombinant porcine factor VIII. The underlying autoimmune disorder frequently responds to immunosuppressive medication. Antiprothrombin antibodies usually are found in patients with antiphospholipid antibody syndrome and can be associated with bleeding. Antibodies of von Willebrand factor are found in patients with type 3 von Willebrand disease in response to infusion of von Willebrand factor. Antibodies to factor V can occur as autoantibodies or as cross-reacting anti-bovine factor V antibodies that develop after exposure to bovine thrombin products that are contaminated with factor V. Pathogenic autoantibodies also have been described that target thrombin, factor IX, factor XI, factor XIII, protein C, protein S and the endothelial cell protein C receptor. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1178755562 ER -