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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Main Authors: Massimo Franchini, Daniele Focosi
Format: Article
Language:English
Published: PAGEPress Publications 2025-01-01
Series:Bleeding, Thrombosis and Vascular Biology
Subjects:
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
description 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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institution Kabale University
issn 2785-5309
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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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