Quintana-Molina M, Martínez-Bahamonde F, González-García E, Romero-Garrido J, Villar-Camacho A, Jiménez-Yuste V, Fernández-Bello I, Hernández-Navarro F
Coagulopathies Centre, Department of Haematology, La Paz University Hospital, Madrid, Spain.
Haemophilia. 2004 Sep;10 Suppl 2:30-40. doi: 10.1111/j.1365-2516.2004.00938.x.
Surgery in haemophilic patients with inhibitor against factor (F)VIII or FIX is high risk. Surgery may be performed with the administration of sufficiently high dose of FVIII in patients with low-response inhibitor or who, despite having a high response, present a low inhibitor titre at the time of surgery. The use of high doses of FX is more complicated in patients with a low-titre FIX inhibitor, as there is a high risk of anaphylactic reactions. In the case of patients with high-titre inhibitors, several treatments have been proposed, such as porcine FVIII, recombinant FVIIa (rFVIIa), and activated prothrombin complex concentrate (APCC). We present our 20 years' experience in the treatment and subsequent management of haemophilic patients with inhibitor in surgery and evaluate the results obtained with the products available for haemostatic control in 64 surgical procedures. The efficacy we obtained with FVIII is good in 100% of the cases described; we had no haemorrhagic complication (HC) in the 18 procedures in which it was used (three major and 15 minor surgery). With APCC we obtained excellent results with only one HC in a synoviorthesis in the form of bleeding and haematomas out of 32 procedures. Good results were obtained with rFVIIa with few haemorrhagic episodes. Thus, in major surgery there was one HC out of three cases. In minor surgery, greater efficacy was observed using extremely large doses of rFVIIa (> or =120 mg kg(-1) 2 h(-1)) because of the shorter half-life of this factor in this type of patients.
对凝血因子(F)VIII或FIX有抑制物的血友病患者进行手术风险很高。对于抑制物反应低的患者,或尽管反应高但在手术时抑制物滴度低的患者,可在给予足够高剂量FVIII的情况下进行手术。对于FIX抑制物滴度低的患者,使用高剂量FX更为复杂,因为存在过敏反应的高风险。对于高滴度抑制物的患者,已提出了几种治疗方法,如猪FVIII、重组FVIIa(rFVIIa)和活化凝血酶原复合物浓缩物(APCC)。我们介绍了在血友病抑制物患者手术治疗及后续管理方面20年的经验,并评估了在64例外科手术中用于止血控制的产品所取得的结果。在所述的所有病例中,我们使用FVIII获得的疗效良好;在使用它的18例手术中(3例大手术和15例小手术),我们没有出现出血并发症(HC)。使用APCC,在32例手术中,仅在1例滑膜切除术中有1例以出血和血肿形式出现的HC,取得了优异的结果。使用rFVIIa取得了良好的结果,出血事件较少。因此,在大手术中,3例中有1例出现HC。在小手术中,由于这类患者中该因子半衰期较短,使用极大剂量的rFVIIa(≥120 mg·kg⁻¹·2 h⁻¹)观察到了更高的疗效。