Hermans Cedric, Gruel Yves, Frenzel Laurent, Krumb Evelien
Haemostasis and Thrombosis Unit, Division of Adult Haematology, Cliniques universitaires Saint-Luc, Université catholique de Louvain (UCLouvain), Avenue Hippocrate 10, 1200 Brussels, Belgium.
Centre Régional de Traitement de l'Hémophilie, Hôpital Trousseau, CHRU de Tours, Tours, France.
Ther Adv Hematol. 2023 Jan 12;14:20406207221145627. doi: 10.1177/20406207221145627. eCollection 2023.
Gene-based therapy opens an entirely new paradigm in managing people with haemophilia (PWH), offering them the possibility of a functional cure by enabling continuous expression of factor VIII (FVIII) or factor IX (FIX) after transfer of a functional gene designed to replace the PWH's own defective gene. In recent years, significant advances in gene therapy have been made, resulting in clotting factor activity attaining near-normal levels, as reflected by 'zero bleeding rates' in previously severely inflicted patients following a single administration of adeno-associated viral (AAV) vectors. While this new approach represents a major advancement, there are still several issues that must be resolved before applying this technology in clinical practice. First, awareness, communication, and education about the therapeutic potential and modalities of gene therapy must be further strengthened. To this end, objective, unbiased, transparent, and regularly updated information must be shared, in an appropriate way and understandable language with the support of patients' organizations. Second, healthcare providers should adopt a patient-centred approach, as the 'one size fits all' approach is inappropriate when considering gene therapy. Instead, a holistic patient view taking into account their physical and mental dimensions, along with unexpressed expectations and preferences, is mandatory. Third, the consent procedure must be improved, ensuring that patients' interests are maximally protected. Finally, gene therapy is likely to be first delivered in a few centres, with the highest expertise and experience in this domain. Thus, patients should be managed based on a hub-and-spoke model, taking into account that the key to gene therapy's success lies in an optimal communication and collaboration both within and between haemophilia centres sharing their experiences in the frame of international registries. This review describes recent progress and explains outstanding hurdles that must be tackled to ease the implementation of this paradigm-changing new therapy.
基于基因的疗法为血友病患者(PWH)的治疗开创了全新的模式,通过导入一个功能性基因来替代患者自身有缺陷的基因,使因子VIII(FVIII)或因子IX(FIX)持续表达,从而为他们提供了实现功能性治愈的可能性。近年来,基因治疗取得了重大进展,凝血因子活性达到了接近正常的水平,这在之前严重出血的患者单次注射腺相关病毒(AAV)载体后出现的“零出血率”中得到了体现。虽然这种新方法代表了一项重大进步,但在将该技术应用于临床实践之前,仍有几个问题必须解决。首先,必须进一步加强对基因治疗的治疗潜力和方式的认识、沟通与教育。为此,必须以适当的方式、用易懂的语言,在患者组织的支持下,分享客观、无偏见、透明且定期更新的信息。其次,医疗服务提供者应采取以患者为中心的方法,因为在考虑基因治疗时,“一刀切”的方法并不合适。相反,必须从整体上看待患者,考虑他们的身心状况以及未表达的期望和偏好。第三,必须改进同意程序,确保最大程度地保护患者的利益。最后,基因治疗可能首先在少数几个在该领域拥有最高专业知识和经验的中心进行。因此,应基于中心辐射模式对患者进行管理,要考虑到基因治疗成功的关键在于血友病中心内部以及之间在国际注册框架内分享经验时的最佳沟通与协作。本综述描述了近期的进展,并解释了在实施这一改变模式的新疗法时必须克服的突出障碍。