[Rinsho ketsueki] The Japanese journal of clinical hematology · 2026
[Autoimmune acquired factor V deficiency developing after thoracic aortic aneurysm repair].
СтатьяКлинический случайАннотация на английскомНазвание и аннотация: Перевод в очереди
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Sasaki Yuya, Murakami Hajime, Harada Takuma, Noguchi Yuma, Oshikawa Gaku
Аннотация
BACKGROUND: Acquired factor V deficiency is a rare bleeding disorder caused by autoantibodies against factor V, resulting in marked reduction of activity and bleeding tendency.
CASE PRESENTATION: We report the case of an 81-year-old man who developed acquired factor V deficiency after total arch replacement and open stent grafting for a thoracic aortic aneurysm. Postoperatively, despite the absence of bleeding tendency, both prothrombin time (PT) and activated partial thromboplastin time (APTT) were markedly prolonged. Factor assays revealed factor V activity <3%, and a factor V inhibitor was detected (1 BU/ml). A cross-mixing test indicated a pattern of factor deficiency, suggesting the involvement of clearance-facilitating antibodies in addition to neutralizing antibodies.
DISCUSSION: Acquired factor V deficiency is often associated with surgery, transfusions, or antibiotics. Although rare, it should be considered in patients with unexplained prolongation of PT and APTT. Cross-mixing tests may mimic factor deficiency patterns when clearance-facilitating antibodies are present, requiring careful interpretation. Immunosuppressive therapy including corticosteroids, cyclophosphamide, or rituximab has been reported effective, while bleeding episodes may be managed with plasma or bypassing agents.
CONCLUSION: This case emphasizes the importance of considering acquired factor V deficiency in the differential diagnosis of postoperative coagulopathy with unexplained prolongation of PT and APTT.
CASE PRESENTATION: We report the case of an 81-year-old man who developed acquired factor V deficiency after total arch replacement and open stent grafting for a thoracic aortic aneurysm. Postoperatively, despite the absence of bleeding tendency, both prothrombin time (PT) and activated partial thromboplastin time (APTT) were markedly prolonged. Factor assays revealed factor V activity <3%, and a factor V inhibitor was detected (1 BU/ml). A cross-mixing test indicated a pattern of factor deficiency, suggesting the involvement of clearance-facilitating antibodies in addition to neutralizing antibodies.
DISCUSSION: Acquired factor V deficiency is often associated with surgery, transfusions, or antibiotics. Although rare, it should be considered in patients with unexplained prolongation of PT and APTT. Cross-mixing tests may mimic factor deficiency patterns when clearance-facilitating antibodies are present, requiring careful interpretation. Immunosuppressive therapy including corticosteroids, cyclophosphamide, or rituximab has been reported effective, while bleeding episodes may be managed with plasma or bypassing agents.
CONCLUSION: This case emphasizes the importance of considering acquired factor V deficiency in the differential diagnosis of postoperative coagulopathy with unexplained prolongation of PT and APTT.