Katarzyna Grabowska, Michalina Grzelak, Lin-Yong Zhao, Elżbieta Płuciennik, Zbigniew Pasieka, Mateusz Kciuk, Adrianna Gielecińska, Aleksander K Smakosz, Żaneta Kałuzińska-Kołat, Damian Kołat
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引用次数: 0
摘要
血友病是一种血浆出血性疾病,其特征是缺乏某些凝血因子。这种疾病最常见的类型是 A 型和 B 型,全球约有 40 万人患有这种疾病。如果不进行适当的治疗,确保适当的凝血级联,这种疾病可能会导致严重的残疾。通过替代疗法可将患者的不适感降至最低,替代疗法包括通过静脉注射替代缺失的凝血因子。频繁用药和因子抑制剂带来的风险是其明显的缺点,因此有必要改进现有疗法或开发新型疗法。本综述探讨了人源化双特异性抗体 Emicizumab,它通过模拟凝血因子 VIII 的作用来确保止血,而凝血因子 VIII 的缺乏会导致 A 型血友病。本文概述了这一主题,然后总结了有关 Emicizumab 治疗 A 型血友病的现有临床试验。多项介入性临床试验发现,在不同的 A 型血友病人群中,Emicizumab 能有效减少出血发作并提高患者满意度。目前与 Emicizumab 相关的试验预计将在 2024 年至 2030 年间完成,除先天性 A 型血友病外,这些试验还包括后天性 A 型血友病和运动型患者。更全面地了解 Emicizumab 可能会彻底改变 A 型血友病的治疗方法,并改善患者的生活质量。最后,Emicizumab 是一种更温和的疗法,因为它采用皮下注射,注射次数更少,从而减少了注射部位反应,减轻了治疗负担,最终降低了医院就诊率和间接成本。
Emicizumab as a Promising Form of Therapy for Type A Hemophilia - A Review of Current Knowledge from Clinical Trials.
Hemophilia is a plasma bleeding disorder characterized by a deficiency of certain blood clotting factors. The most common forms of this disease, i.e., type A and type B, affect approximately 400,000 people worldwide. Without appropriate treatment ensuring the proper coagulation cascade, this disease may lead to serious disability. Minimizing patient discomfort is possible via replacement therapy, consisting of the substitution of a missing coagulation factor via intravenous administration. Frequent medication and the risk related to factor inhibitors are significant disadvantages, necessitating the improvement of current therapies or the development of novel ones. This review examines the humanized bispecific antibody Emicizumab which ensures hemostasis by mimicking the action of the coagulation factor VIII, a deficiency of which causes type A hemophilia. The paper outlines the topic and then summarizes available clinical trials on Emicizumab in type A hemophilia. Several interventional clinical trials have found Emicizumab to be effective in decreasing bleeding episodes and raising patient satisfaction among various hemophilia A populations. Current Emicizumab-related trials are forecast to be completed between 2024 and 2030, and in addition to congenital hemophilia A, the trials cover acquired hemophilia A and patients playing sports. Providing a more comprehensive understanding of Emicizumab may revolutionize the management of hemophilia type A and improve quality of life. Conclusively, Emicizumab is a gentler therapy owing to subcutaneous delivery and fewer injections, which reduces injection-site reactions and makes therapy less burdensome, ultimately decreasing hospital visits and indirect costs.
期刊介绍:
Current Protein & Peptide Science publishes full-length/mini review articles on specific aspects involving proteins, peptides, and interactions between the enzymes, the binding interactions of hormones and their receptors; the properties of transcription factors and other molecules that regulate gene expression; the reactions leading to the immune response; the process of signal transduction; the structure and function of proteins involved in the cytoskeleton and molecular motors; the properties of membrane channels and transporters; and the generation and storage of metabolic energy. In addition, reviews of experimental studies of protein folding and design are given special emphasis. Manuscripts submitted to Current Protein and Peptide Science should cover a field by discussing research from the leading laboratories in a field and should pose questions for future studies. Original papers, research articles and letter articles/short communications are not considered for publication in Current Protein & Peptide Science.