Zhang Ai Hong, Skupsky Jonathan, Scott David W
Department of Surgery, and Center for Vascular and Inflammatory Diseases, University of Maryland School of Medicine, 800 West Baltimore Street, Room 319, Baltimore, MD 21201, USA.
Clin Rev Allergy Immunol. 2009 Oct;37(2):114-24. doi: 10.1007/s12016-009-8122-5.
Patients with hemophilia A are deficient in coagulation Factor VIII. This bleeding disorder can be treated with Factor VIII replacement therapy, but close to a third of patients will be immunized to the treatment and begin to form inhibitory antibodies known as "inhibitors". These inhibitors will render the treatment ineffective and represent the most severe complication in the treatment of hemophilia A. In this review, we highlight factors involved in inhibitor development and emphasize research being done to modulate the immune response to this life-saving therapy.
甲型血友病患者缺乏凝血因子VIII。这种出血性疾病可用因子VIII替代疗法治疗,但近三分之一的患者会对该治疗产生免疫反应,并开始形成被称为“抑制剂”的抑制性抗体。这些抑制剂会使治疗无效,是甲型血友病治疗中最严重的并发症。在本综述中,我们强调了参与抑制剂形成的因素,并着重介绍了为调节对这种挽救生命疗法的免疫反应而开展的研究。