Abstract
Background
Acquired Hemophilia A (AHA) is a rare autoimmune disorder associated with the development
of autoantibodies against factor VIII (FVIII). Although obtaining hemostatic control
through the use of recombinant factor VIIa, activated prothrombin complex concentrate
and recombinant porcine FVIII are cornerstones in the clinical management of AHA,
these therapies have several disadvantages, including a higher risk for the development
of thromboembolic events, unpredictable efficacy and short half-lives. While emicizumab
has been FDA licensed for use in bleeding prophylaxis for patients with Congenital
Hemophilia A (CHA) with and without inhibitors, it has not been approved for use in
AHA, with only a few reports describing its use in this context.
Case report
We report our experience with the use of emicizumab in an 83-year old male with AHA,
complicated by the onset of atrial fibrillation following admission, drug-induced
thrombocytopenia, infectious complications, and the identification of a low-grade
lymphoproliferative disorder, in which emicizumab prophylaxis was used for bleeding
prophylaxis in the context of persistently elevated inhibitor titers without evidence
of thrombotic events or thrombotic microangiopathy.
Keywords
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Article info
Publication history
Published online: May 11, 2022
Accepted:
May 9,
2022
Received in revised form:
April 30,
2022
Received:
March 6,
2022
Identification
Copyright
© 2022 Published by Elsevier Ltd.
