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Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable. Methods. The patient's plasma wa...

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Autores principales: Abt, Nicholas B., Streiff, Michael B., Gocke, Christian B., Kickler, Thomas S., Lanzkron, Sophie M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4052897/
https://www.ncbi.nlm.nih.gov/pubmed/24955264
http://dx.doi.org/10.1155/2014/484563
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author Abt, Nicholas B.
Streiff, Michael B.
Gocke, Christian B.
Kickler, Thomas S.
Lanzkron, Sophie M.
author_facet Abt, Nicholas B.
Streiff, Michael B.
Gocke, Christian B.
Kickler, Thomas S.
Lanzkron, Sophie M.
author_sort Abt, Nicholas B.
collection PubMed
description Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable. Methods. The patient's plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours. Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity. Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered.
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spelling pubmed-40528972014-06-22 Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor Abt, Nicholas B. Streiff, Michael B. Gocke, Christian B. Kickler, Thomas S. Lanzkron, Sophie M. Case Rep Hematol Case Report Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable. Methods. The patient's plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours. Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity. Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered. Hindawi Publishing Corporation 2014 2014-05-14 /pmc/articles/PMC4052897/ /pubmed/24955264 http://dx.doi.org/10.1155/2014/484563 Text en Copyright © 2014 Nicholas B. Abt et al. 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 Case Report
Abt, Nicholas B.
Streiff, Michael B.
Gocke, Christian B.
Kickler, Thomas S.
Lanzkron, Sophie M.
Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor
title Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor
title_full Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor
title_fullStr Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor
title_full_unstemmed Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor
title_short Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor
title_sort idiopathic acquired hemophilia a with undetectable factor viii inhibitor
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4052897/
https://www.ncbi.nlm.nih.gov/pubmed/24955264
http://dx.doi.org/10.1155/2014/484563
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