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[A long-lasting idiopathic factor V inhibitor].

Yasushi Okoshi¹, Hideki Akiyama, Takashi Inoue

¹Hematology Division, Tokyo Metropolitan Komagome Hospital.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology

|March 26, 2003

Summary

This case report details a rare, long-standing idiopathic acquired blood coagulation factor V inhibitor. Treatment with immunosuppressants showed partial success, highlighting the challenges in managing this uncommon bleeding disorder.

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Area of Science:

Hematology
Immunology

Background:

Acquired factor V inhibitors are rare autoimmune disorders causing significant bleeding.
Idiopathic acquired factor V inhibitors, without an underlying cause, are even rarer.

Observation:

A 78-year-old male presented with hematuria and melena due to a factor V inhibitor with FV activity <1%.
Mixing studies confirmed an inhibitor, and Western blot identified anti-FV antibodies.

Findings:

Initial treatment with fresh-frozen plasma, platelets, and methylprednisolone provided partial correction of coagulation parameters.
Disease exacerbation upon steroid dose tapering necessitated cyclophosphamide treatment.

Implications:

This case highlights the diagnostic and therapeutic challenges of prolonged idiopathic acquired factor V inhibitors.

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The rarity and potential for relapse underscore the need for tailored, long-term management strategies.