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Pharmacotherapy of haemophilia A.

机译：A型血友病的药物治疗。

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INTRODUCTION: Haemophilia A is due to factor VIII (FVIII) deficiency. The main treatment is replacement therapy with FVIII concentrates. However, these concentrates carried a high risk of blood-borne viral infections and still have a high risk of inducing anti-FVIII inhibitors. AREAS COVERED: An overview of products available and therapeutic options for haemophilia A management in order to help in decision making. A literature search using Medline with the keywords: 'haemophilia', 'factor VIII', 'therapy', 'inhibitor', 'concentrate', 'bleeding', 'prophylaxis', 'on demand', 'plasma-derived', 'recombinant', 'coagulation factors', 'immunotolerance' was performed. The years 1960 - 2010 are included. EXPERT OPINION: Progress in management of patients with haemophilia A has allowed increased life expectancy and quality of life. There is evidence that prophylaxis prevents or, at least, slows down arthropathy development when started early in childhood. FVIII concentrates have achieved high levels of blood-borne pathogen safety. However, treatment is frequently complicated by development of FVIII-neutralizing inhibitors, which prevent control of bleeding and predispose to a high morbidity and mortality risk. Bypassing agents are effective in bleeding treatment in a high percentage of cases. Prophylaxis with bypassing agents and their use in combination are offering opportunities in management of inhibitor patients. More evidence is necessary to understand how to prevent and manage this complication.
机译：简介：血友病A是由于VIII因子（FVIII）缺乏引起的。主要治疗方法是用FVIII浓缩液替代治疗。但是，这些浓缩物具有血源性病毒感染的高风险，并且仍然具有诱导抗FVIII抑制剂的高风险。覆盖的区域：血友病A管理的可用产品和治疗选择的概述，以帮助决策。使用Medline搜索具有以下关键词的文献：'血友病'，'因子VIII'，'治疗'，'抑制剂'，'浓缩'，'出血'，'预防'，'按需'，'血浆来源'，'进行“重组”，“凝血因子”，“免疫耐受”。包括1960年至2010年。专家意见：甲型血友病患者管理的进步使预期寿命和生活质量得到了提高。有证据表明，预防措施至少在儿童时期就开始阻止或减缓了关节炎的发展。 FVIII浓缩物已实现高水平的血源性病原体安全性。但是，开发FVIII中和抑制剂通常会使治疗复杂化，这会阻止出血的控制，并容易导致高发病率和死亡风险。旁路剂在很多情况下可有效治疗出血。绕行剂的预防及其组合使用为抑制剂患者的治疗提供了机会。需要更多证据来了解如何预防和管理这种并发症。

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