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Mysterious bruises
1Department of Medicine, Kingsbrook Jewish Medical Center, Brooklyn, NY, USA.
Journal of Postgraduate Medicine
|October 2, 2012
Summary
This case study details a 69-year-old man with acquired hemophilia A and pernicious anemia. Treatment with steroids and rituximab successfully eradicated the factor VIII inhibitor.
Area of Science:
- Hematology
- Immunology
Background:
- Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII.
- It can present with spontaneous, severe bleeding and requires prompt diagnosis and management.
Observation:
- A 69-year-old male presented with spontaneous bruising and hematomas.
- Initial labs showed prolonged activated partial thromboplastin time (aPTT) with normal platelet count and international normalized ratio.
- Factor VIII inhibitor was detected, confirming AHA.
Findings:
- The patient also had pernicious anemia, an autoimmune condition.
- Initial treatment with steroids normalized aPTT and undetectable factor VIII inhibitor levels.
- A relapse occurred, necessitating treatment with recombinant factor VIIa, steroids, and rituximab.
Implications:
- This case highlights the importance of a thorough workup for bleeding disorders, including the investigation of associated autoimmune conditions.
- Combined immunosuppressive therapy (steroids and rituximab) can be effective in eradicating factor VIII inhibitors in AHA.
- Early recognition and multidisciplinary management are crucial for favorable outcomes in acquired hemophilia A.
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