Sunagawa T, Uezu Y, Kadena K, Tokuyama K, Kinjo F, Saito A
First Department of Internal Medicine, Faculty of Medicine, University of the Ryukyus, Nishihara, Okinawa, Japan.
Br J Haematol. 1999 Mar;104(3):465-7. doi: 10.1046/j.1365-2141.1999.01220.x.
Factor VIII (FVIII) inhibitors appear in 3-20% of haemophilia A patients after injection of FVIII concentrates. However, autoantibodies to FVIII are also reported in non-haemophiliacs. In these patients FVIII inhibitor disappears spontaneously or diminishes in response to immunosuppressive therapy. However, a few patients show resistance to immunosuppressive therapy. We describe a non-haemophilic elderly patient with acquired FVIII inhibitor who failed to respond to prednisolone. He was treated with double-filtration plasmapheresis (DFPP) which resulted in a very rapid reduction in inhibitor levels and resolution of symptoms.
在注射凝血因子VIII(FVIII)浓缩物后,3%至20%的甲型血友病患者会出现FVIII抑制剂。然而,非血友病患者中也有针对FVIII的自身抗体的报道。在这些患者中,FVIII抑制剂会自发消失或在免疫抑制治疗后减少。然而,少数患者对免疫抑制治疗有抵抗性。我们描述了一名患有获得性FVIII抑制剂的非血友病老年患者,其对泼尼松龙治疗无反应。他接受了双重滤过血浆置换术(DFPP),结果抑制剂水平迅速降低,症状得到缓解。