Ljung Rolf C R
Department of Clinical Sciences - Paediatrics, Lund University, Lund, Sweden.
Centre for Thrombosis and Haemostasis, Skåne University Hospital Malmö, Malmo, Sweden.
Br J Haematol. 2018 Feb;180(4):501-510. doi: 10.1111/bjh.15053. Epub 2017 Dec 22.
Development of inhibitors to coagulation factor VIII or IX is still the most challenging complication in haemophilia care. 'Bypassing agents' may be used to treat a bleed but the eradication of the inhibitor by immune tolerance induction (ITI) is the main objective in the treatment of a patient with haemophilia who has developed neutralizing antibodies. Several options exist for ITI and the patient may be at 'good' or 'bad risk' for successful outcome with different regimens. This paper offers a review of current regimens to be considered in the treatment of a bleed in a patient with an inhibitor but the main focus is the aspects of different choices in the management of the child or the adult with severe or mild forms of haemophilia A or B, who has developed an inhibitor. There are also some final outlooks on new and emerging treatment possibilities.
开发凝血因子VIII或IX的抑制剂仍然是血友病治疗中最具挑战性的并发症。“旁路制剂”可用于治疗出血，但通过免疫耐受诱导（ITI）消除抑制剂是治疗已产生中和抗体的血友病患者的主要目标。ITI有几种选择，不同治疗方案下患者成功治愈的风险可能为“高”或“低”。本文综述了目前用于治疗有抑制剂的患者出血的方案，但主要关注的是患有严重或轻度血友病A或B且已产生抑制剂的儿童或成人在治疗管理中不同选择的方面。此外，还对新出现的治疗可能性进行了一些最终展望。
