Department of Internal Medicine, American University of Beirut, Naef K. Basile Cancer Institute, Beirut, Lebanon.
Department of Hematology and Medical Oncology, Emory University School of Medicine, Winship Cancer Institute, Atlanta, Georgia.
Cancer. 2018 Oct 15;124(20):3979-3989. doi: 10.1002/cncr.31550. Epub 2018 Jun 15.
Myelodysplastic syndromes (MDS) encompass a heterogeneous group of clonal hematopoietic stem cell disorders characterized by a broad clinical spectrum related to ineffective hematopoiesis leading to unilineage or multilineage cytopenias, with a high propensity for transformation to acute myeloid leukemia. Iron overload has been recently identified as one of the important conditions complicating the management of these diverse disorders. The accumulation of iron is mainly related to chronic transfusions; however, evidence suggests a possible role for ineffective erythropoiesis and increased intestinal absorption of iron, related to altered hepcidin and growth differentiation factor-15 levels in the development of hemosiderosis in patients with MDS. In addition to its suggested role in the exacerbation of ineffective erythropoiesis, multiple reports have identified a prognostic implication for the development of iron overload in patients with MDS, with an improvement in overall survival after the initiation of iron chelation therapy. This review includes a detailed discussion of iron overload in patients with MDS whether they are undergoing supportive therapy or curative hematopoietic stem cell transplantation, with a focus on the mechanism, diagnosis, and effect on survival as well as the optimal management of this highly variable complication.
骨髓增生异常综合征(MDS)是一组异质性克隆性造血干细胞疾病,其特征为无效造血导致单系或多系细胞减少,具有向急性髓系白血病转化的高倾向,临床表现谱广泛。铁过载最近被确定为影响这些不同疾病治疗的重要因素之一。铁的积累主要与慢性输血有关;然而,有证据表明无效性红细胞生成和铁吸收增加可能与 MDS 患者铁蛋白和生长分化因子-15 水平改变有关。除了其在无效性红细胞生成恶化中的作用外,多项报告还确定了 MDS 患者铁过载的预后意义,铁螯合治疗开始后总生存率提高。本文详细讨论了接受支持治疗或根治性造血干细胞移植的 MDS 患者的铁过载问题,重点讨论了其发病机制、诊断、对生存的影响以及这种高度可变并发症的最佳治疗方法。