Autores: Mingot-Castellano ME, Pardos-Gea J, Marco Rico A, Alcalde-Mellado P, Salvadores-Alvares V, Jordano Jimenes A, Canaro Hirnyk M, Gutierrez AB, Bastida JM, Rodriguez Alén JA, et al. (…)
DOI: 10.3324/haematol.2025.288480
Publicado online: 14 de agosto de 2025
Article
Acquired hemophilia (AH) is a rare autoimmune bleeding disorder caused by spontaneous development of autoantibodies against any protein coagulation factor, but the most frequent ones are antibodies against factor VIII (AHA). AHA can affect both men and women, with a higher prevalence in elderly patients and women at the postpartum period. It is often associated with underlying conditions such as autoimmune diseases, postpartum or cancer. The main symptom of AHA are spontaneous bleedings, with life-threatening bleedings in close to 70% of this patients. Treatment focuses on controlling bleeding episodes and eliminating the autoantibodies. Hemostatic agents, such as activated recombinant factor VII (rFVIIa) or activated prothrombin complex concentrates (aPCC) are used to stop bleedings. Recently, emicizumab, a bispecific antibody that mimics the function of FVIIIa intrinsic tenase complex, is being used as hemostatic prophylaxis in this group of patients. To eliminate inhibitors, immunosuppressive therapy is used, including steroids, cyclophosphamide and rituximab. (…)
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