Terumo Blood and Cell Technologies, Ikaroslaan 41, Zaventem 1930, Belgium.
Centre Hospitalier Intercommunal de Créteil, France.
Transfus Apher Sci. 2024 Oct;63(5):103988. doi: 10.1016/j.transci.2024.103988. Epub 2024 Aug 8.
In France, sickle cell disease (SCD) is the most common rare disease and represents the most prevalent genetic disorder, with 19,800 to 32,400 patients diagnosed in 2016 and 1:714 newborns affected in 2019. SCD is caused by a single mutation in the β-globin gene, resulting in the production of abnormal hemoglobin (called HbS), chronic hemolytic anemia, and impaired red blood cell rheology. SCD patients face several severe acute and chronic complications, including stroke, acute chest syndrome (ACS), painful vaso-occlusive crisis (VOC), organ failure, and a high risk of infections. As patients' care pathway remains unclear in France, a roundtable advisory board meeting was organized in the country to provide insights into the management of SCD in alignment with clinical guidelines. The meeting brought together a panel of esteemed key opinion leaders (KOLs) in SCD management, encompassing both clinical practice and research. During the meeting, the KOLs discussed clinical practices and their alignment with French guidelines, identifying areas of concordance and discrepancy. They also addressed disparities in SCD clinical practices across regions and medical centers. The KOLs discussed the prophylactic and therapeutic options currently available for SCD patients in France, with a focus on transfusion therapies, especially automated red blood cell exchange (aRBCX). The results of this advisory board meeting provide a valuable platform for gathering expert perspectives on SCD management, clinical practices, guideline alignment, and the potential for contributions to guideline updates.
在法国,镰状细胞病(SCD)是最常见的罕见病,也是最常见的遗传性疾病,2016 年诊断出的患者有 19800 至 32400 例,2019 年受影响的新生儿有 1:714。SCD 是由β-珠蛋白基因中的单个突变引起的,导致产生异常血红蛋白(称为 HbS)、慢性溶血性贫血和红细胞流变性受损。SCD 患者面临多种严重的急性和慢性并发症,包括中风、急性胸部综合征(ACS)、疼痛性血管阻塞性危象(VOC)、器官衰竭和感染风险高。由于法国的患者护理路径仍不明确,因此在该国组织了一次圆桌顾问委员会会议,以根据临床指南深入了解 SCD 的管理。会议汇集了一组在 SCD 管理方面享有盛誉的关键意见领袖(KOL),涵盖临床实践和研究。在会议期间,KOL 讨论了与法国指南一致的临床实践及其一致性和差异。他们还讨论了法国不同地区和医疗中心之间 SCD 临床实践的差异。KOL 讨论了法国目前 SCD 患者可用的预防和治疗选择,重点是输血疗法,尤其是自动化红细胞交换(aRBCX)。此次顾问委员会会议的结果为汇集 SCD 管理、临床实践、指南一致性以及为指南更新做出贡献的专家意见提供了宝贵的平台。