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Acquired haemophilia A (AHA) is a rare but potentially life-threatening bleeding disorder characterized by autoantibodies directed against coagulation factor VIII.These autoantibodies constitute the most common spontaneous inhibitor to any coagulation factor and may induce spontaneous bleeding in patients with no previous history of a bleeding disorder.
Bypassing agents such as recombinant FVIIa can be used in acquired hemophilia. Antibody formation to factor VIII can also be a major concern for patients receiving therapy against bleeding; the incidence of these inhibitors is dependent of various factors, including the factor VIII product itself. [23]
People may develop antibodies to factor VIII such that this medication becomes less effective. [15] Factor VIII was first identified in the 1940s and became available as a medication in the 1960s. [16] [17] Recombinant factor VIII was first made in 1984 and approved for medical use in the United States in 1992.
In moderate haemophilia clotting factors are typically only needed when bleeding occurs or to prevent bleeding with certain events. [16] In severe haemophilia preventive use is often recommended two or three times a week and may continue for life. [16] Rapid treatment of bleeding episodes decreases damage to the body. [16]
Concizumab, sold under the brand name Alhemo, is a monoclonal antibody used for the treatment of hemophilia A and hemophilia B. [5] [8] It is an anti-tissue factor pathway inhibitor. [5] [8] The most common adverse reactions include injection site reactions and hives (urticaria). [11]
Hemophilia is a family of rare genetic blood diseases caused by a clotting factor deficiency (FVIII in hemophilia A, FIX in hemophilia B), impacting more than 800,000 people globally.
Monoclonal antibody emicizumab has been approved by the FDA in 2017 for therapy of hemophilia A. [28] In July 2024, a recent study published in the New England Journal of Medicine demonstrated that efanesoctocog alfa , a bioengineered human factor VIII recombinant protein, prophylaxis for children with severe hemophilia A could have therapeutic ...
These inhibitors often increase over time and inhibit the action of coagulation in the body. Recombinant factor VIIa, which is an activated form of factor VII, bypasses factors VIII and IX and causes coagulation without the need for factors VIII and IX. It may be used in acquired hemophilia patients with higher inhibitor titers. [15]
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