Jacobs Jeremy W, Amorim Luiz, Pirenne France, Tayou Claude, Adimora Ijele, Pecker Lydia H, Tobian Aaron A R, Callum Jeannie, Makani Julie, Gladwin Mark T, Delaney Meghan, Triulzi Darrell J, Odame Isaac, Bloch Evan M
Department of Pathology, Microbiology, & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
HEMORIO, Instituto Estadual de Hematologia, Rio de Janeiro, Brazil.
Br J Haematol. 2025 Jun;206(6):1585-1592. doi: 10.1111/bjh.20055. Epub 2025 Mar 27.
Globally, sickle cell disease (SCD) is the most common inherited haemoglobinopathy. The highest burden of SCD is encountered in low- and middle-income countries (LMICs), most of which lack the resources to contend with the disease. There is a marked divide between care for individuals with SCD in high-income countries (HICs) versus LMICs, whereby the few disease-modifying therapies and curative regimens are only accessible to those in HICs. As such, blood transfusion remains central to the emergent treatment and prevention of complications of SCD. However, there are a myriad of related challenges in LMICs, which have impeded efforts to treat patients with SCD effectively. In addition to blood safety and availability, examples that impact SCD specifically include capabilities to detect and/or manage red blood cell alloimmunization, capacity for automated red cell exchange, limited immunohematology, suboptimal quality oversight with a lack of safeguards to prevent transfusion of incompatible blood and limited or absent post-transfusion surveillance to detect and/or manage transfusion-associated adverse events. Consequently, clinical practices that are otherwise regarded as standard of care in HICs remain the exception in LMICs, highlighting disparities in care. A multifaceted approach that prioritizes transfusion support in LMICs is needed to improve care for patients with SCD.
在全球范围内,镰状细胞病(SCD)是最常见的遗传性血红蛋白病。低收入和中等收入国家(LMICs)面临着最高的SCD负担,其中大多数国家缺乏应对该疾病的资源。高收入国家(HICs)与LMICs对SCD患者的护理存在明显差异,只有HICs的患者能够获得少数疾病改善疗法和治愈方案。因此,输血仍然是SCD紧急治疗和预防并发症的核心。然而,LMICs存在无数相关挑战,阻碍了有效治疗SCD患者的努力。除了血液安全和供应外,具体影响SCD的因素还包括检测和/或管理红细胞同种免疫的能力、自动红细胞置换的能力、有限的免疫血液学、缺乏防止输注不相容血液的保障措施的次优质量监督,以及检测和/或管理输血相关不良事件的有限或缺乏输血后监测。因此,在HICs被视为标准护理的临床实践在LMICs中仍然是例外,凸显了护理方面的差异。需要一种多方面的方法,优先在LMICs中提供输血支持,以改善对SCD患者的护理。