ACQUIRED HAEMOPHILIA A: AN INTRIGUING DISEASE ACQUIRED HAEMOPHILIA A
Main Article Content
Keywords
: autoantibodies against FVIII, bleeding symptoms, bleeding treatment, eradication therapy.
Abstract
Acquired Haemophilia A is a rare acquired bleeding disorder caused by autoantibodies directed against Factor VIII, which neutralize FVIII activity. These inhibitors differ from alloantibodies against FVIII which can occur in congenital Haemophilia A after repeated exposures to plasma-derived or recombinant FVIII products. In most cases the disease occurs suddenly in subjects without personal or familiar history of bleedings, with symptoms that may be mild, moderate or severe. However, only laboratory alterations are present in ? 30% of patients. The incidence varies from 1 to 4 cases per million/year; more than 80% of patients are elderly, males and females are similarly affected. There is a small peak of incidence related to pregnancy in young women aged 20–40 years. The disease may be underdiagnosed in the elderly. The diagnostic algorithm is based on an isolated prolonged activated partial thromboplastin time, normal thrombin time, absence of Lupus Anticoagulant and a mixing test that reveals the presence of an inhibitor: the finding of reduced FVIII activity and the detection of neutralising autoantibodies against FVIII lead to diagnosis. The disease is idiopathic in 44%-63% of cases, while in the others etiological factors are present. Bleeding prevention and treatment are based on therapeutic tools as bypassing agents, recombinant porcine FVIII concentrate or, in a limited number of cases, FVIII concentrates and desmopressin. As soon as the diagnosis has been made, immunosuppressive therapy must be started to eradicate the inhibitor. Better knowledge of the disease, optimal management of bleeding and eradication of the inhibitor have significantly reduced morbidity and mortality in most patients.
Keywords: autoantibodies against FVIII, bleeding symptoms, bleeding treatment, eradication therapy.
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References
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7) Knoebl P, Marco P, Baudo F, Collins P, Huth-Ku¨ hne A, Nemes L, Pellegrini F, Tengborn L, Le´ vesque H, on behalf of the EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).
J Thromb Haemost 2012; 10: 622–31.
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10) M Franchini and G Lippi. Acquired factor VIII inhibitors. Blood; 2008;112 :250-255
11) J Charlebois, G -É Rivarda, J n St-Louis Management of acquired hemophilia A: Review of current evidence. Transfusion and Apheresis Science 57; 2018 717–720
12) Franchini M, Targher G, Montagnana M, Lippi G. Laboratory, clinical and therapeutic aspects of acquired haemophilia A. Clin Chim Acta 2008; 395: 14-18
13) Collins PW, Percy CL. Advances in the understanding of acquired haemophilia A: implications for clinical practice. Br J Haematol 2010; 148: 183-194.
14) Verbruggen B, et al. The Nijmegen modification of the Bethesda assay for factor VIII:C inhibitors: improved specificity and reliability. Thromb Haemost 1995; 73: 247-251.
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