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加强非洲镰状细胞病的先进治疗:坦桑尼亚达累斯萨拉姆镰状细胞病中心的经验

Strengthening advanced therapy for sickle cell disease in Africa: experience from sickle cell disease centre in Dar es Salaam, Tanzania.

作者信息

Bukini Daima, Rifai Aisha, Kanza Collin, Luoga Fredrick, Maingu Deogratius, Kassim Kassim, Mashaka Jennifer, Kisali Eka Patricia, Karim Salmaan, Alimohamed Mohamed Zahir, Manongi Janeth, Lema Winfrida, Chuwa Harrison, Konteh Sisawo, Urio Florence, Minja Irene Kida, Balandya Emmanuel, Moshi Grace, Makani Julie

机构信息

Sickle Cell Programme, Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania

Johns Hopkins University Berman Institute of Bioethics, Baltimore, Maryland, USA.

出版信息

BMJ Glob Health. 2025 Jan 27;10(1):e017878. doi: 10.1136/bmjgh-2024-017878.

Abstract

Despite progress in healthcare services for individuals living with sickle cell disease (SCD) in Africa, substantial gaps remain in advanced treatments for SCD. To help address this burden, Tanzania has established one of the largest single-centre SCD programmes in the world and developed an advanced therapy programme for SCD focused on patient engagement and advocacy, clinical activities involving exchange blood transfusion (ExBT) and haematopoietic stem cell transplant (HSCT), gene therapy (GT) preparedness, and enabling partnerships. This report describes the programme's genesis, structure and progress achieved. Patient engagement camps and patient-focused workshops conducted since early 2021 have involved more than 150 patients, family caregivers and healthcare providers. A patient registry was established by screening 1500 patients eligible for advanced therapies with 157 identified to benefit from advanced treatments for SCD. Out of which 22 patients received ExBT, and human leucocyte antigen typing was conducted on 127 individuals to establish a registry of family members with potential to be HSCT donors. Target product profiles were devised for minimum and optimum criteria of GT products to guide drug discovery and development efforts, and qualitative research was conducted to investigate factors anticipated to influence successful adoption of GTs for SCD in Africa. The programme's multifaceted components have been enabled by institutional networks and collaborations established at national, regional and global levels. The programme presented opportunities to deliver cost-effective advanced treatment and curative options for SCD in Tanzania and lessons learnt may be applicable to inform similar efforts in other African regions where SCD is highly endemic.

摘要

尽管非洲镰状细胞病(SCD)患者的医疗服务取得了进展,但SCD的先进治疗仍存在巨大差距。为了帮助减轻这一负担,坦桑尼亚建立了世界上最大的单中心SCD项目之一,并制定了一项针对SCD的先进治疗项目,重点是患者参与和宣传、涉及换血输血(ExBT)和造血干细胞移植(HSCT)的临床活动、基因治疗(GT)准备以及建立合作关系。本报告描述了该项目的起源、结构和取得的进展。自2021年初以来举办的患者参与营和以患者为中心的研讨会吸引了150多名患者、家庭护理人员和医疗服务提供者。通过对1500名符合先进治疗条件的患者进行筛查,建立了一个患者登记册,其中157人被确定可从SCD的先进治疗中受益。其中22名患者接受了ExBT,并对127人进行了人类白细胞抗原分型,以建立有潜力成为HSCT供体的家庭成员登记册。为GT产品的最低和最佳标准设计了目标产品概况,以指导药物研发工作,并进行了定性研究,以调查预计会影响GT在非洲成功应用于SCD的因素。该项目的多方面组成部分得益于在国家、区域和全球层面建立的机构网络和合作。该项目为在坦桑尼亚提供具有成本效益的SCD先进治疗和治愈方案提供了机会,所吸取的经验教训可能适用于为SCD高度流行的其他非洲地区的类似努力提供参考。

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