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Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder
Acquired hemophilia A (AHA) is a rare hemorrhagic disease in which autoantibodies against coagulation factor VIII- (FVIII-) neutralizing antibodies (inhibitors) impair the intrinsic coagulation system. As the inhibitors developed in AHA are autoantibodies, the disease may have an autoimmune cause an...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3987878/ https://www.ncbi.nlm.nih.gov/pubmed/24741588 http://dx.doi.org/10.1155/2014/320674 |
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author | Sakurai, Yoshihiko Takeda, Tomohiro |
author_facet | Sakurai, Yoshihiko Takeda, Tomohiro |
author_sort | Sakurai, Yoshihiko |
collection | PubMed |
description | Acquired hemophilia A (AHA) is a rare hemorrhagic disease in which autoantibodies against coagulation factor VIII- (FVIII-) neutralizing antibodies (inhibitors) impair the intrinsic coagulation system. As the inhibitors developed in AHA are autoantibodies, the disease may have an autoimmune cause and is often associated with autoimmune disease. Although acute hemorrhage associated with AHA may be fatal and is costly to treat, AHA is often unrecognized or misdiagnosed. AHA should thus be considered in the differential diagnosis particularly in postpartum women and the elderly with bleeding tendency or prolonged activated partial thromboplastin time. Cross-mixing tests and measurement of FVIII-binding antibodies are useful to confirm AHA diagnosis. For treatment of acute hemorrhage, hemostatic therapy with bypassing agents should be provided. Unlike in congenital hemophilia A with inhibitors, in which immune tolerance induction therapy using repetitive infusions of high-dose FVIII concentrates is effective for inhibitor eradication, immune tolerance induction therapy has shown poor efficacy in treating AHA. Immunosuppressive treatment should thus be initiated to eradicate inhibitors as soon as the diagnosis of AHA is confirmed. |
format | Online Article Text |
id | pubmed-3987878 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-39878782014-04-16 Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder Sakurai, Yoshihiko Takeda, Tomohiro J Immunol Res Review Article Acquired hemophilia A (AHA) is a rare hemorrhagic disease in which autoantibodies against coagulation factor VIII- (FVIII-) neutralizing antibodies (inhibitors) impair the intrinsic coagulation system. As the inhibitors developed in AHA are autoantibodies, the disease may have an autoimmune cause and is often associated with autoimmune disease. Although acute hemorrhage associated with AHA may be fatal and is costly to treat, AHA is often unrecognized or misdiagnosed. AHA should thus be considered in the differential diagnosis particularly in postpartum women and the elderly with bleeding tendency or prolonged activated partial thromboplastin time. Cross-mixing tests and measurement of FVIII-binding antibodies are useful to confirm AHA diagnosis. For treatment of acute hemorrhage, hemostatic therapy with bypassing agents should be provided. Unlike in congenital hemophilia A with inhibitors, in which immune tolerance induction therapy using repetitive infusions of high-dose FVIII concentrates is effective for inhibitor eradication, immune tolerance induction therapy has shown poor efficacy in treating AHA. Immunosuppressive treatment should thus be initiated to eradicate inhibitors as soon as the diagnosis of AHA is confirmed. Hindawi Publishing Corporation 2014 2014-03-24 /pmc/articles/PMC3987878/ /pubmed/24741588 http://dx.doi.org/10.1155/2014/320674 Text en Copyright © 2014 Y. Sakurai and T. Takeda. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Sakurai, Yoshihiko Takeda, Tomohiro Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder |
title | Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder |
title_full | Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder |
title_fullStr | Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder |
title_full_unstemmed | Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder |
title_short | Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder |
title_sort | acquired hemophilia a: a frequently overlooked autoimmune hemorrhagic disorder |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3987878/ https://www.ncbi.nlm.nih.gov/pubmed/24741588 http://dx.doi.org/10.1155/2014/320674 |
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