DiMichele Donna
Pediatrics and Public Health, Weill Medical College of Cornell University, New York, NY, USA.
Br J Haematol. 2007 Aug;138(3):305-15. doi: 10.1111/j.1365-2141.2007.06657.x.
Factor IX (FIX) inhibitors develop in 1.5-3% of haemophilia B patients. Due to its low incidence compared with that in haemophilia A, few comparable data exist on host and treatment-related risk factors, and immunological processes associated with FIX inhibitor development. Moreover, the safety and efficacy of bypass therapy as well as the outcome predictors of successful inhibitor eradication have been poorly characterised. The lack of a useful evidence-based approach to the diagnosis and management of FIX inhibitors complicates their significant morbidity due to the frequency of allergic reactions that often herald antibody development. This review discusses what is currently known about the epidemiology, natural history and immunology of anti-FIX antibody development. It addresses several special considerations in the approach to the treatment of bleeding and inhibitor eradication. A case is made for moving forward with an integrated international collaboration for the further study of the nature and treatment of this problem.
在1.5%至3%的乙型血友病患者中会产生FIX（凝血因子IX）抑制剂。由于与甲型血友病相比其发病率较低，关于宿主和治疗相关危险因素以及与FIX抑制剂产生相关的免疫过程的可比数据很少。此外，旁路治疗的安全性和有效性以及成功根除抑制剂的结果预测因素尚未得到充分描述。由于过敏反应频繁（这往往预示着抗体的产生），缺乏基于证据的有效方法来诊断和管理FIX抑制剂，使得它们的显著发病率情况更为复杂。本综述讨论了目前已知的关于抗FIX抗体产生的流行病学、自然史和免疫学知识。它阐述了在治疗出血和根除抑制剂的方法中的几个特殊考虑因素。提出了开展国际综合合作以进一步研究这个问题的本质和治疗方法的理由。
