emicizumab (hemlibra°) in haemophilia A-A first-choice preventive treatment for patients with factor VIII inhibitors

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emicizumab (hemlibra°) in haemophilia A-A first-choice preventive treatment for patients with factor VIII inhibitors

机译：Emicizumab（Hemlibra°）在血友病A-A患者VIII抑制剂患者的首选预防治疗中

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Haemophilia A is an inherited bleeding disorder due to factorVIII deficiency. Patients with moderate or severe haemophilia A are treated by replacing the deficient factorVIII. However, factor VIII administration induces the production of factor VIII inhibitors (anti-factor VIII antibodies) in some patients, rendering the treatment ineffective. In this situation, one option is to use a "bypassing" agent (activated factor VII or activated pro-thrombin complex), administered "on demand" or as continuous prophylaxis. Another option is an "immune tolerance induction" protocol, in which high doses of factor VIII are administered daily or several times a week for several months or several years, often successfully suppressing the production of factorVIII inhibitors.

机译：血友病A是因子缺乏因子缺乏而遗传的出血障碍。通过替代缺陷的因子综合症治疗患有中度或严重的血友病患者。然而，因子VIII施用在一些患者中诱导因子VIII抑制剂（抗因子VIII抗体），使治疗无效。在这种情况下，一种选择是使用“旁路”试剂（活性因子VII或活化的促凝血酶复合物），以“按需”或作为连续预防施用。另一种选择是“免疫耐受诱导”方案，其中每天施用高剂量的因子VIII或每周施用几个月或几年，通常成功地抑制因子硫含量的产生。

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《Prescrire international》 |2019年第210期|共1页
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1. emicizumab (hemlibra°) in haemophilia A-A first-choice preventive treatment for patients with factor VIII inhibitors [J] . Prescrire international . 2019,第210期

机译：Emicizumab（Hemlibra°）在血友病A-A患者VIII抑制剂患者的首选预防治疗中
2. Management of bleeding and invasive procedures in haemophilia A patients with inhibitor treated with emicizumab (Hemlibra ? ? ): Proposals from the French network on inherited bleeding disorders (MHEMO), the French Reference Centre on Haemophilia, in collaboration with the French Working Group on Perioperative Haemostasis (GIHP) [J] . Susen Sophie, Gruel Yves, Godier Anne, Haemophilia: the official journal of the World Federation of Hemophilia . 2019,第5期

机译：血友病患者的出血和侵入性手术治疗emicizumab（Hemlibra？）患者：来自法国网络上遗传性出血障碍（MHEMO）的提案，法国嗜血症对血友病的法国参考中心，与法国工作组进行围攻血统（GIHP）
3. Hemlibra((R)) (Emicizumab) receives EU Approval for Routine Prophylaxis of Hemophilia A with Inhibitors against Coagulation Factor VIII [J] . Transfusion medicine and hemotherapy: offizielles Organ der Deutschen Gesellschaft fur? Transfusionsmedizin und Immunham?atologie . 2018,第2期

机译：Hemlibra（（r））（emicizumab）获得欧盟批准血友病常规预防，血友病A与凝血因子VIII抑制剂
4. Case Report: Successful Treatment of a Spontaneous Acquired inhibitor Against Factor VIII in a 73-Year-old Patient [C] . R. Klamroth, F. Seibt, S. Gottstein Hemophilie-Symposion . 2004

机译：病例报告：在73岁的患者中成功治疗反对因子VIII的自发获得的抑制剂
5. Combined deficiency of factors V and VIII by chance coinheritance of parahaemophilia and haemophilia A, but not by mutations of either LMAN1 or MCFD2 [D] . 鈴木幸子 2019

机译：副血友病和A血友病的偶然一致性导致因子V和VIII的联合缺乏，但不是LMAN1或MCFD2的突变
6. Treatment and Prevention of Bleeds in Haemophilia Patients with Inhibitors to Factor VIII/IX [O] . Angiola Rocino, Massimo Franchini, Antonio Coppola 2017

机译：凝血因子VIII / IX抑制剂对血友病患者出血的治疗和预防
7. PSY155 - DETERMINING THE VALUE OF EMICIZUMAB (HEMLIBRA®) FOR THE PROPHYLAXIS TREATMENT OF HAEMOPHILIA A PATIENTS WITH INHIBITORS IN SPAIN BY MULTI-CRITERIA DECISION ANALYSIS (MCDA) [O] . M.T. Álvarez, I. Cuervo-Arango, R. Perez-Santamarina, 2018

机译：PSY155 - 通过多标准决策分析（MCDA）确定西班牙抑制剂患者的预防治疗Emicizumab（Hemlibra®）的值
8. Cooperative Study of Spontaneously-Occurring Factor VIII Inhibitors in Patients with Hemophilia A [R] . Shapiro, S. S. 1981

机译：血友病a患者自发性因子VIII抑制剂的合作研究

emicizumab (hemlibra°) in haemophilia A-A first-choice preventive treatment for patients with factor VIII inhibitors

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