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[血友病治疗的现状与未来展望]

[Current status and future perspective of treatment for hemophilia].

作者信息

Kashiwakura Yuji, Ohmori Tsukasa

机构信息

Department of Biochemistry, Jichi Medical University School of Medicine.

出版信息

Rinsho Ketsueki. 2023;64(5):377-388. doi: 10.11406/rinketsu.64.377.

Abstract

Hemophiliacs are X-linked inherited bleeding disorders. The treatment of hemophilia is based on periodic replacement treatment with coagulation factor products to avoid the development of hemophilic arthropathy, as well as to control fatal bleeding. However, because of the coagulation factor's incredibly short half-life, the medication must be often given intravenously throughout one's life. Extended half-life coagulation factor preparations have lowered the burden of treatment by enhancing pharmacokinetics. However, the patients remain at risk of developing inhibitors to significantly interfere with the therapeutic effectiveness of coagulation factor concentrates. Recently, nonfactor replacement therapies have gained a lot of interest as a new class of therapeutic drugs preventing bleeding in patients with or without inhibitors. Moreover, gene therapy drugs for hemophilia have recently been approved in Europe and the United States. Because gene therapy can preserve coagulation factor levels in the blood for at least several years with a single treatment, it may become a curative treatment that removes the need for the therapy for a long time.

摘要

血友病是X连锁遗传性出血性疾病。血友病的治疗基于定期使用凝血因子产品进行替代治疗,以避免血友病性关节病的发展,并控制致命性出血。然而,由于凝血因子的半衰期极短,患者一生中必须经常静脉给药。延长半衰期的凝血因子制剂通过增强药代动力学降低了治疗负担。然而,患者仍有产生抑制剂的风险,这会显著干扰凝血因子浓缩物的治疗效果。最近,非因子替代疗法作为一类预防有或无抑制剂患者出血的新型治疗药物受到了广泛关注。此外,血友病基因治疗药物最近已在欧洲和美国获批。由于基因治疗单次治疗就能使血液中的凝血因子水平维持至少数年,它可能成为一种无需长期治疗的治愈性疗法。

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