White G C, Roberts H R
Center for Thrombosis and Hemostasis, University of North Carolina, Chapel Hill, 27599-7035, USA.
Vox Sang. 1996;70 Suppl 1:19-23.
The management of patients with classic hemophilia who develop inhibitory antibodies against transfused factor VIII has been the frequent subject of international and national conferences. While treatment of such patients cannot be considered as standardized, several therapeutic approaches are currently available. These include the induction of immune tolerance, removal or eradication of inhibitory antibodies by immune adsorption and/or immune suppression, neutralization of the inhibitor by porcine factor VIII or high doses of human factor VIII, and bypassing of the inhibitor using recombinant factor VIIa or other bypassing agents. In addition to currently available therapeutic strategies, other approaches are under investigation, including the use of selected factor VIII peptides that block the inhibitor; the use of immunologically mute factor VIII products, for example a human-porcine factor VIII chimera not recognized by factor VIII inhibitors, and the use of anti-idiotypic antibodies. The ability to recognize those patients destined to develop inhibitors is also under active investigation.
患有经典血友病且对输注的凝血因子 VIII 产生抑制性抗体的患者的管理一直是国际和国内会议经常讨论的主题。虽然对此类患者的治疗不能被视为标准化,但目前有几种治疗方法可供选择。这些方法包括诱导免疫耐受、通过免疫吸附和/或免疫抑制去除或消除抑制性抗体、用猪凝血因子 VIII 或高剂量人凝血因子 VIII 中和抑制剂,以及使用重组凝血因子 VIIa 或其他旁路制剂绕过抑制剂。除了目前可用的治疗策略外,其他方法也在研究中,包括使用可阻断抑制剂的特定凝血因子 VIII 肽;使用免疫惰性的凝血因子 VIII 产品,例如不被凝血因子 VIII 抑制剂识别的人 - 猪凝血因子 VIII 嵌合体,以及使用抗独特型抗体。识别那些注定会产生抑制剂的患者的能力也在积极研究中。