Hematology and Hemotherapy Center, University of Campinas - UNICAMP, Campinas, Brazil.
Hematology and Hemotherapy Center, University of Campinas - UNICAMP, Campinas, Brazil; Division of Hematology, University of Washington, Seattle, WA.
Transfus Med Rev. 2019 Jul;33(3):170-175. doi: 10.1016/j.tmrv.2019.04.001. Epub 2019 May 2.
Sickle cell disease (SCD) is a frequent indication for chronic transfusion, which can cause iron overload. Excess iron often affects the liver, but not the heart in SCD. Magnetic resonance (MR) is recommended to detect myocardial iron overload (MIO) but its elevated cost requires optimized indication. We aimed to compile all published data on MIO in SCD upon the description of a fatal case of severe MIO in our institution, and to determine associated risk factors. We performed a systematic review using the PRISMA guidelines in two databases (PubMed and Web of Science). Inclusion criteria were publication in English, patients diagnosed with SCD, and reporting ferritin and MIO by MR. Twenty publications reported on 865 SCD adult and pediatric patients, with at least 10 other cases of MIO. The prevalence of MIO in chronically transfused SCD patients can be estimated to be 3% or less, and is associated with high transfusion burden, top-up transfusions, and low adherence to iron chelation. Cardiac siderosis in SCD is rarely reported, and increased awareness with better use of the available screening tools are necessary. Prospective studies should define the recommended chelation regimens depending on the severity of MIO.
镰状细胞病 (SCD) 是慢性输血的常见指征，这可能导致铁过载。过量的铁通常会影响肝脏，但不会影响 SCD 中的心脏。磁共振 (MR) 被推荐用于检测心肌铁过载 (MIO)，但由于其高昂的成本，需要优化其指征。在我们机构发生一例严重 MIO 的致死性病例后，我们旨在编译所有关于 SCD 中 MIO 的已发表数据，并确定相关的危险因素。我们使用 PRISMA 指南在两个数据库 (PubMed 和 Web of Science) 中进行了系统评价。纳入标准为英文发表、诊断为 SCD 且通过 MR 报告铁蛋白和 MIO 的患者。有 20 篇出版物报道了 865 例 SCD 成年和儿科患者，其中至少有 10 例其他 MIO 病例。慢性输血的 SCD 患者中 MIO 的患病率估计为 3%或更低，与高输血负担、补充输血和低铁螯合依从性有关。SCD 中心脏铁沉着症很少见，需要提高认识并更好地使用现有筛查工具。应根据 MIO 的严重程度定义推荐的螯合方案。
