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Severe bleeding caused by an inhibitor to coagulation factor V: a case report
W M Smid1, J T de Wolf, J H Nijland
1Department of Haematology, University Hospital Groningen, The Netherlands.
Summary
A high level of factor V inhibitor caused severe bleeding in a patient. Prolonged high-dose steroid therapy eventually eliminated the inhibitor, though steroid dependence persisted.
Area of Science:
- Hematology
- Immunology
Background:
- Acquired factor V inhibitors are rare but can cause severe, life-threatening bleeding.
- Management of factor V inhibitors is challenging due to variable responses to immunosuppressive therapies.
Observation:
- A 67-year-old male presented with severe bleeding secondary to a high titer (350 BU) factor V inhibitor.
- Initial treatment with prednisolone and cyclophosphamide led to transient improvement in coagulation abnormalities.
Findings:
- Subsequent monotherapy with cyclophosphamide or cyclosporine was ineffective in controlling the inhibitor.
- Prolonged high-dose steroid therapy over two years resulted in undetectable factor V inhibitor levels.
- The patient remained dependent on steroids to maintain remission.
Implications:
- This case highlights the potential efficacy of prolonged high-dose steroid therapy for refractory factor V inhibitors.
- It also underscores the challenges in achieving complete remission and the risk of long-term steroid dependence.
- Optimal therapeutic strategies for factor V inhibitors require individualized approaches and long-term monitoring.