The changing landscape of treatment for acquired hemophilia A
Acquired hemophilia A (AHA) is a rare acquired autoimmune disorder caused by autoantibodies against coagulation factor VIII (FVIII), which cause a hemorrhagic diathesis, not rarely of severe degree. Standard treatment consists of bleeding control with bypassing agents (recombinant activated factor...
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PAGEPress Publications
2025-01-01
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Series: | Bleeding, Thrombosis and Vascular Biology |
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Online Access: | https://www.btvb.org/btvb/article/view/157 |
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author | Massimo Franchini Daniele Focosi |
author_facet | Massimo Franchini Daniele Focosi |
author_sort | Massimo Franchini |
collection | DOAJ |
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Acquired hemophilia A (AHA) is a rare acquired autoimmune disorder caused by autoantibodies against coagulation factor VIII (FVIII), which cause a hemorrhagic diathesis, not rarely of severe degree. Standard treatment consists of bleeding control with bypassing agents (recombinant activated factor VII and activated prothrombin complex concentrate) and recombinant porcine FVIII, and immunosuppressive therapy (corticosteroids with or without cyclophosphamide). Recent studies have renewed the interest towards the immunosuppressive agent rituximab for FVIII inhibitor eradication and have suggested a potential role for emicizumab for the prevention of bleeding in AHA patients. This narrative review will focus on the placement of these two emerging drugs within the treatment landscape for AHA.
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format | Article |
id | doaj-art-ea67099cb0944cd4bc388657e6da64f9 |
institution | Kabale University |
issn | 2785-5309 |
language | English |
publishDate | 2025-01-01 |
publisher | PAGEPress Publications |
record_format | Article |
series | Bleeding, Thrombosis and Vascular Biology |
spelling | doaj-art-ea67099cb0944cd4bc388657e6da64f92025-01-30T09:04:03ZengPAGEPress PublicationsBleeding, Thrombosis and Vascular Biology2785-53092025-01-014110.4081/btvb.2025.157The changing landscape of treatment for acquired hemophilia AMassimo Franchini0https://orcid.org/0000-0002-8795-0580Daniele Focosi1https://orcid.org/0000-0001-8811-195XDepartment of Transfusion Medicine and Hematology, Carlo Poma Hospital, MantuaNorth-Western Tuscany Blood Bank, Pisa University Hospital, Pisa Acquired hemophilia A (AHA) is a rare acquired autoimmune disorder caused by autoantibodies against coagulation factor VIII (FVIII), which cause a hemorrhagic diathesis, not rarely of severe degree. Standard treatment consists of bleeding control with bypassing agents (recombinant activated factor VII and activated prothrombin complex concentrate) and recombinant porcine FVIII, and immunosuppressive therapy (corticosteroids with or without cyclophosphamide). Recent studies have renewed the interest towards the immunosuppressive agent rituximab for FVIII inhibitor eradication and have suggested a potential role for emicizumab for the prevention of bleeding in AHA patients. This narrative review will focus on the placement of these two emerging drugs within the treatment landscape for AHA. https://www.btvb.org/btvb/article/view/157Acquired Hemophilia AFVIII inhibitorrituximabemicizumab |
spellingShingle | Massimo Franchini Daniele Focosi The changing landscape of treatment for acquired hemophilia A Bleeding, Thrombosis and Vascular Biology Acquired Hemophilia A FVIII inhibitor rituximab emicizumab |
title | The changing landscape of treatment for acquired hemophilia A |
title_full | The changing landscape of treatment for acquired hemophilia A |
title_fullStr | The changing landscape of treatment for acquired hemophilia A |
title_full_unstemmed | The changing landscape of treatment for acquired hemophilia A |
title_short | The changing landscape of treatment for acquired hemophilia A |
title_sort | changing landscape of treatment for acquired hemophilia a |
topic | Acquired Hemophilia A FVIII inhibitor rituximab emicizumab |
url | https://www.btvb.org/btvb/article/view/157 |
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