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Acquired Hemophilia A and urothelial carcinoma
Acquired Hemophilia A (AHA) is a rare entity, resulting from the production of autoantibodies against Factor VIII of the coagulation cascade. These autoantibodies may develop in response to autoimmune conditions, drugs, neoplastic diseases, and pregnancy. Diagnosis involves clinical presentation, mu...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Taylor & Francis
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7850392/ https://www.ncbi.nlm.nih.gov/pubmed/33552425 http://dx.doi.org/10.1080/20009666.2020.1836726 |
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author | Taza, Fadi Suleman, Nawar Paz, Robert Haas, Christopher |
author_facet | Taza, Fadi Suleman, Nawar Paz, Robert Haas, Christopher |
author_sort | Taza, Fadi |
collection | PubMed |
description | Acquired Hemophilia A (AHA) is a rare entity, resulting from the production of autoantibodies against Factor VIII of the coagulation cascade. These autoantibodies may develop in response to autoimmune conditions, drugs, neoplastic diseases, and pregnancy. Diagnosis involves clinical presentation, mucocutaneous or intramuscular bleeding, and laboratory findings, such as prolonged activated partial thromboplastin time, decreased levels of Factor VIII, and the presence of Factor VIII autoantibodies. The etiology is diverse, with a variety of underlying culprits. Malignancy-associated AHA has been associated with approximately 15% of cases. Urothelial malignancy-mediated AHA is exceedingly rare, with only two previously published reports. The management of AHA includes stabilization and control of bleeding via the use of hemostatic agents, and elimination of the inhibitor with immunosuppressive therapy. Here, we report a case of AHA secondary to urothelial malignancy and review the pathobiology and pathogenesis of Hemophilia A and AHA. |
format | Online Article Text |
id | pubmed-7850392 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Taylor & Francis |
record_format | MEDLINE/PubMed |
spelling | pubmed-78503922021-02-05 Acquired Hemophilia A and urothelial carcinoma Taza, Fadi Suleman, Nawar Paz, Robert Haas, Christopher J Community Hosp Intern Med Perspect Case Report Acquired Hemophilia A (AHA) is a rare entity, resulting from the production of autoantibodies against Factor VIII of the coagulation cascade. These autoantibodies may develop in response to autoimmune conditions, drugs, neoplastic diseases, and pregnancy. Diagnosis involves clinical presentation, mucocutaneous or intramuscular bleeding, and laboratory findings, such as prolonged activated partial thromboplastin time, decreased levels of Factor VIII, and the presence of Factor VIII autoantibodies. The etiology is diverse, with a variety of underlying culprits. Malignancy-associated AHA has been associated with approximately 15% of cases. Urothelial malignancy-mediated AHA is exceedingly rare, with only two previously published reports. The management of AHA includes stabilization and control of bleeding via the use of hemostatic agents, and elimination of the inhibitor with immunosuppressive therapy. Here, we report a case of AHA secondary to urothelial malignancy and review the pathobiology and pathogenesis of Hemophilia A and AHA. Taylor & Francis 2021-01-26 /pmc/articles/PMC7850392/ /pubmed/33552425 http://dx.doi.org/10.1080/20009666.2020.1836726 Text en © 2020 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group on behalf of Greater Baltimore Medical Center. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Taza, Fadi Suleman, Nawar Paz, Robert Haas, Christopher Acquired Hemophilia A and urothelial carcinoma |
title | Acquired Hemophilia A and urothelial carcinoma |
title_full | Acquired Hemophilia A and urothelial carcinoma |
title_fullStr | Acquired Hemophilia A and urothelial carcinoma |
title_full_unstemmed | Acquired Hemophilia A and urothelial carcinoma |
title_short | Acquired Hemophilia A and urothelial carcinoma |
title_sort | acquired hemophilia a and urothelial carcinoma |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7850392/ https://www.ncbi.nlm.nih.gov/pubmed/33552425 http://dx.doi.org/10.1080/20009666.2020.1836726 |
work_keys_str_mv | AT tazafadi acquiredhemophiliaaandurothelialcarcinoma AT sulemannawar acquiredhemophiliaaandurothelialcarcinoma AT pazrobert acquiredhemophiliaaandurothelialcarcinoma AT haaschristopher acquiredhemophiliaaandurothelialcarcinoma |