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A Case of Acquired Factor V Deficiency Treated with Corticosteroids and Cyclophosphamide

Steroid와 Cyclophosphamide로 치료한 후천성 제5인자 결핍증

  • Ha, Sung-Hae (Department of Internal Medicine, Bundang Jesaeng General Hospital, Daejin Medical Center) ;
  • Kim, Jung-Hee (Department of Internal Medicine, Bundang Jesaeng General Hospital, Daejin Medical Center) ;
  • Jang, Sung-Yeol (Department of Internal Medicine, Bundang Jesaeng General Hospital, Daejin Medical Center) ;
  • Hwang, Jeong-Ah (Department of Internal Medicine, Bundang Jesaeng General Hospital, Daejin Medical Center) ;
  • Sohn, Hee-Jung (Department of Internal Medicine, Bundang Jesaeng General Hospital, Daejin Medical Center) ;
  • Yeon, Jae-Woo (Department of Radiology, Bundang Jesaeng General Hospital, Daejin Medical Center)
  • Published : 2012.01.01

Abstract

Acquired factor V deficiency is a rare bleeding disorder, the severity of which ranges from mild to fatal. There are various suggested treatments, including transfusion of fresh frozen plasma (FFP) or platelets, plasmapheresis and immunosuppressive therapy. We encountered a case of idiopathic acquired factor V deficiency with fatal retroperitoneal bleeding treated with steroid and cyclophosphamide.

후천성 제5인자 결핍증은 드문 출혈성 질환으로, 무증상에서부터 생명을 위협하는 중증 출혈을 유발하기도 한다. 치료로는 신선냉동혈장 및 혈소판 수혈, 혈장분리교환술, 면역억제요법 등이 이용되고 있다. 저자들은 특발성 후천성 제5인자 결핍증으로 후복막강 출혈이 발생한 환자에게 steroid와 cyclophosphamide를 투여하여 성공적으로 치료하였으며, 이는 국내에서 보고된 예가 없기에 문헌고찰과 함께 보고하는 바이다.

Keywords

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  2. Factor V Deficiency in Korean Patients: Clinical and Laboratory Features, Treatment, and Outcome vol.31, pp.2, 2012, https://doi.org/10.3346/jkms.2016.31.2.208
  3. A Case of Acquired Factor V Deficiency after Percutaneous Coronary Intervention vol.92, pp.5, 2012, https://doi.org/10.3904/kjm.2017.92.5.480