Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor
Joint Authors
Kickler, Thomas S.
Gocke, Christian B.
Lanzkron, Sophie M.
Abt, Nicholas B.
Streiff, Michael B.
Source
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-05-13
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Abt, Nicholas B.& Streiff, Michael B.& Gocke, Christian B.& Kickler, Thomas S.& Lanzkron, Sophie M.. 2014. Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor. Case Reports in Hematology،Vol. 2014, no. 2014, pp.1-4.
https://search.emarefa.net/detail/BIM-475298
Modern Language Association (MLA)
Abt, Nicholas B.…[et al.]. Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor. Case Reports in Hematology No. 2014 (2014), pp.1-4.
https://search.emarefa.net/detail/BIM-475298
American Medical Association (AMA)
Abt, Nicholas B.& Streiff, Michael B.& Gocke, Christian B.& Kickler, Thomas S.& Lanzkron, Sophie M.. Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor. Case Reports in Hematology. 2014. Vol. 2014, no. 2014, pp.1-4.
https://search.emarefa.net/detail/BIM-475298
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-475298