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Acquired hemophilia: a case report
Mariusz Flisiński1, Jerzy Windyga, Ewa Stefańska
1Department of Nephrology, Hypertension and Internal Diseases, Collegium Medicum of Nicolaus Copernicus University, Bydgoszcz, Poland. nerka@nerka.cpro.pl
Polskie Archiwum Medycyny Wewnetrznej
|June 26, 2008
Summary
Acquired hemophilia, an autoimmune disorder, can cause severe bleeding. This case highlights successful treatment of acquired hemophilia A with immunosuppression, leading to complete inhibitor removal and normalized coagulation.
Area of Science:
- Hematology
- Immunology
- Nephrology
Background:
- Acquired hemophilia A (AHA) is a rare, life-threatening bleeding disorder caused by autoantibodies against coagulation factor VIII.
- It presents with spontaneous or trauma-induced bleeding, often severe, affecting various patient demographics.
Observation:
- A 54-year-old woman presented with acute renal failure and urinary tract bleeding attributed to idiopathic AHA.
- Diagnostic tests revealed significantly prolonged activated partial thromboplastin time (APTT), a high factor VIII inhibitor titer (121 BU/ml), and severely reduced factor VIII activity (2%).
Findings:
- Treatment involved hemostatic support with prothrombin complex concentrates and immunosuppression using prednisone and cyclophosphamide.
- This regimen successfully reduced the factor VIII inhibitor titer to 38 BU/ml and increased factor VIII activity to 4% initially.
- Long-term treatment over two years normalized APTT and factor VIII activity to 108%, eradicating the inhibitor.
Implications:
- This case demonstrates the efficacy of combined hemostatic and immunosuppressive therapy in achieving long-term remission of acquired hemophilia A.
- Successful management can restore normal hemostasis and prevent life-threatening bleeding complications, including renal failure.
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