Rare Case Report: Acquired Factor V Deficiency With an Idiopathic Inhibitor

後天性第五凝血因子缺乏症為一種罕見疾病，成因為第五因子抗體的形成，抗體的種類大部分未明，其他如接觸牛型蛋白物質、抗生素、感染、惡性腫瘤、自體免疫疾病、妊娠、輸血或妊娠等也可能產生抗體。最適合的治療方式目前尚無定論，血漿置換術為較少被報導的有效治療方式之一。此病例報告描述一位臨床上診斷為後天性第五凝血因子缺乏症的婦人，使用冷凍新鮮血漿、血小板和類固醇都無法有效改善其凝血功能異常，最後是以血漿置換術成功改善其急性胃腸道出血和凝血功能異常。建議臨床上可考慮使用血漿置換術控制此類病患的急性出血事件，臨床上亦需要後續的研究以確立其最合適的交換血漿容量和治療時間。

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並列摘要

Acquired factor V deficiency is a rare disorder caused by inhibitor formation of idiopathic origin, bovine protein exposure, antibiotics, infection, malignancy, autoimmune disease, transfusion, or pregnancy. The disorder has variable clinical manifestations. The optimal treatment modality of acquired factor V deficiency is not fully determined. The use of plasmapheresis, a suggested treatment, is seldom reported. We herein describe the case of an 83-year-old woman with incident impression of acquired factor V deficiency with an idiopathic inhibitor. Her coagulopathy responded poorly to fresh frozen plasma, single-donor platelets, and steroids. Active gastrointestinal bleeding was noted and supposed by acquired factor V deficiency during admission. Plasmapheresis resolved her active bleeding and coagulopathy. Clinicians should consider plasmapheresis when treating acute haemorrhagic events in patients with acquired factor V deficiency. Further investigation is required to establish the optimal dose and treatment course of plasmapheresis.

並列關鍵字

Acquired factor V deficiency ； Idiopathic factor V inhibitor ； Plasmapheresis

國際替代計量

Rare Case Report: Acquired Factor V Deficiency With an Idiopathic Inhibitor

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