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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...

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Detalles Bibliográficos
Autores principales: Taza, Fadi, Suleman, Nawar, Paz, Robert, Haas, Christopher
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taylor & Francis 2021
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
Descripción
Sumario: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.