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Acquired Factor VIII Inhibitors: Three Cases

Acquired hemophilia A is a rare, but devastating bleeding disorder caused by spontaneous development of autoantibodies directed against coagulation factor VIII. In 40%-50% of patients it is associated with such conditions as the postpartum period, malignancy, use of medications, and autoimmune disea...

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Autores principales: Kyaw, Tay Za, Jayaranee, S., Bee, Ping Chong, Chin, Edmund Fui Min
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Galenos Publishing 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3781661/
https://www.ncbi.nlm.nih.gov/pubmed/24385759
http://dx.doi.org/10.4274/tjh.2012.0009
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author Kyaw, Tay Za
Jayaranee, S.
Bee, Ping Chong
Chin, Edmund Fui Min
author_facet Kyaw, Tay Za
Jayaranee, S.
Bee, Ping Chong
Chin, Edmund Fui Min
author_sort Kyaw, Tay Za
collection PubMed
description Acquired hemophilia A is a rare, but devastating bleeding disorder caused by spontaneous development of autoantibodies directed against coagulation factor VIII. In 40%-50% of patients it is associated with such conditions as the postpartum period, malignancy, use of medications, and autoimmune diseases; however, its cause is unknown in most cases. Acquired hemophilia A should be suspected in patients that present with a coagulation abnormality, and a negative personal and family history of bleeding. Herein we report 3 patients with acquired hemophilia A that had different underlying pathologies, clinical presentations, and therapeutic responses. Factor VIII inhibitor formation in case 1 occurred 6 months after giving birth; underlying disorders were not identified in cases 2 or 3. The bleeding phenotype in these patients’ ranged from no bleeding tendency with isolated prolongation of APTT (activated partial thromboplastin time) to severe intramuscular hematoma and hemarthrosis necessitating recombinant activated factor VII infusion and blood components transfusion. Variable responses to immunosuppressive treatment were also observed. Conflict of interest:None declared.
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spelling pubmed-37816612014-01-02 Acquired Factor VIII Inhibitors: Three Cases Kyaw, Tay Za Jayaranee, S. Bee, Ping Chong Chin, Edmund Fui Min Turk J Haematol Case Report Acquired hemophilia A is a rare, but devastating bleeding disorder caused by spontaneous development of autoantibodies directed against coagulation factor VIII. In 40%-50% of patients it is associated with such conditions as the postpartum period, malignancy, use of medications, and autoimmune diseases; however, its cause is unknown in most cases. Acquired hemophilia A should be suspected in patients that present with a coagulation abnormality, and a negative personal and family history of bleeding. Herein we report 3 patients with acquired hemophilia A that had different underlying pathologies, clinical presentations, and therapeutic responses. Factor VIII inhibitor formation in case 1 occurred 6 months after giving birth; underlying disorders were not identified in cases 2 or 3. The bleeding phenotype in these patients’ ranged from no bleeding tendency with isolated prolongation of APTT (activated partial thromboplastin time) to severe intramuscular hematoma and hemarthrosis necessitating recombinant activated factor VII infusion and blood components transfusion. Variable responses to immunosuppressive treatment were also observed. Conflict of interest:None declared. Galenos Publishing 2013-03 2013-03-05 /pmc/articles/PMC3781661/ /pubmed/24385759 http://dx.doi.org/10.4274/tjh.2012.0009 Text en © Turkish Journal of Hematology, Published by Galenos Publishing. http://creativecommons.org/licenses/by/2.5/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kyaw, Tay Za
Jayaranee, S.
Bee, Ping Chong
Chin, Edmund Fui Min
Acquired Factor VIII Inhibitors: Three Cases
title Acquired Factor VIII Inhibitors: Three Cases
title_full Acquired Factor VIII Inhibitors: Three Cases
title_fullStr Acquired Factor VIII Inhibitors: Three Cases
title_full_unstemmed Acquired Factor VIII Inhibitors: Three Cases
title_short Acquired Factor VIII Inhibitors: Three Cases
title_sort acquired factor viii inhibitors: three cases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3781661/
https://www.ncbi.nlm.nih.gov/pubmed/24385759
http://dx.doi.org/10.4274/tjh.2012.0009
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