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[Autoimmune acquired factor V deficiency developing after thoracic aortic aneurysm repair].
Yuya Sasaki1, Hajime Murakami1, Takuma Harada1
1Department of Hematology, Japanese Red Cross Musashino Hospital.
Summary
Acquired factor V deficiency, a rare bleeding disorder, can occur post-surgery. This case highlights its importance in diagnosing unexplained prolonged clotting times (PT and APTT) after procedures.
Area of Science:
- Hematology
- Immunology
Background:
- Acquired factor V deficiency is a rare bleeding disorder.
- It is caused by autoantibodies against factor V, leading to reduced activity and bleeding.
- This condition is occasionally linked to surgery, transfusions, or antibiotic use.
Purpose of the Study:
- To report a case of acquired factor V deficiency in an elderly patient following thoracic aortic aneurysm repair.
- To discuss the diagnostic challenges and implications of this rare disorder.
Main Methods:
- Case report of an 81-year-old male.
- Postoperative monitoring of coagulation parameters (PT, APTT).
- Factor V activity assays and inhibitor detection.
- Cross-mixing tests to differentiate antibody types.
Main Results:
- The patient developed markedly prolonged PT and APTT post-surgery despite no bleeding.
- Factor V activity was <3% with a detectable factor V inhibitor.
- Cross-mixing tests suggested both neutralizing and clearance-facilitating antibodies.
Conclusions:
- Acquired factor V deficiency should be considered in postoperative coagulopathy with unexplained prolonged PT and APTT.
- Cross-mixing tests require careful interpretation due to potential clearance-facilitating antibodies.
- Effective treatments include immunosuppression and management of bleeding episodes.
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