Scharf R, Kucharski W, Nowak T
Department of Surgery, Institute of Hematology and Blood Transfusion, 5 Chocimska Street, 00-957 Warsaw, Poland.
World J Surg. 1996 Nov-Dec;20(9):1171-81. doi: 10.1007/s002689900179.
Patients with hemophilia A and circulating anticoagulant (factor VIII inhibitor) present a difficult, even unsolvable problem, particularly if they require surgical treatment and the inhibitor titer is high. During the 1986-1995 period 29 surgical procedures on inhibitor hemophilia A patients were performed in our center. Each of the cases had an individual character, and all demanded special clinical treatment. Based on this experience we present the possibilities of hemostasis maintenance during the perioperative period with high doses of human or porcine factor VIII, aPCC, plasmapheresis, and extracorporeal antibody adsorption to protein A-Sepharose. In some patients hemostasis maintenance requires combined treatment. To induce immunotolerance in patients with inhibitor is the gold standard treatment because it is then possible to achieve proper hemostasis after factor VIII infusion. Various methods of immunotolerance induction have been discussed and compared with our experience with immunotolerance induction in 11 patients with small factor VIII doses (25 IU/kg twice a week) and the modified Malmö protocol in 15 patients.
患有甲型血友病且体内存在循环抗凝剂（Ⅷ因子抑制剂）的患者面临着一个棘手甚至难以解决的问题，尤其是当他们需要接受外科治疗且抑制剂滴度很高时。在1986年至1995年期间，我们中心对患有抑制剂的甲型血友病患者进行了29例外科手术。每个病例都有其独特之处，并且都需要特殊的临床治疗。基于这一经验，我们介绍了在围手术期通过大剂量人或猪源性Ⅷ因子、活化凝血酶原复合物（aPCC）、血浆置换以及体外抗体吸附到蛋白A-琼脂糖上来维持止血的可能性。在一些患者中，维持止血需要联合治疗。诱导抑制剂患者产生免疫耐受是金标准治疗，因为这样在输注Ⅷ因子后就有可能实现适当的止血。已经讨论了各种诱导免疫耐受的方法，并与我们对11例小剂量Ⅷ因子（每周两次，25 IU/kg）诱导免疫耐受的经验以及15例患者采用改良的马尔默方案进行了比较。
