Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, the Netherlands.
Haemophilia. 2014 May;20 Suppl 4:76-9. doi: 10.1111/hae.12404.
Inhibitor development is a serious complication of treatment with coagulation products. Presently, 25-30% of all newly diagnosed patients with severe haemophilia A are diagnosed with inhibitors. An increasing number of genetic and non-genetic risk factors have been reported to be involved, although the impact of them in understanding the aetiology is still limited. Much attention has been focused on factor VIII products, but more recent studies show very little, if any, difference between class plasma and recombinant factor VIII products. More intensive treatment and higher dosing are probably more important factors. More than 10% of the inhibitors diagnosed in the last decade are of low titre. A first goal should be to understand their importance. It is argued that the impact of different risk factors should be studied in high-titre inhibitors to prevent dilution by non-significant low-titre inhibitors.
抑制剂的产生是凝血因子治疗的严重并发症。目前，所有新诊断的重度甲型血友病患者中，有 25%-30%被诊断为存在抑制剂。尽管它们在了解病因方面的影响仍然有限，但越来越多的遗传和非遗传风险因素已被报道与之相关。人们已经关注了因子 VIII 产品，但最近的研究表明，类血浆和重组因子 VIII 产品之间几乎没有差异。更密集的治疗和更高的剂量可能是更重要的因素。在过去十年中诊断出的抑制剂中，有超过 10%的抑制剂效价较低。首要目标应该是了解它们的重要性。有人认为，应该在高滴度抑制剂中研究不同风险因素的影响，以防止被非显著低滴度抑制剂稀释。
