Angelucci Emanuele, Cianciulli Paolo, Finelli Carlo, Mecucci Cristina, Voso Maria Teresa, Tura Sante
U.O. Ematologia Ospedale Policlinico San Martino, Largo Rosanna Benzi 10, 16132, Genova, Italy.
Independent clinical iron expert, via E.L. Cerva 200, Rome, Italy.
Leuk Res. 2017 Nov;62:108-115. doi: 10.1016/j.leukres.2017.10.001. Epub 2017 Oct 5.
Myelodysplastic syndromes (MDS) are a group of clonally-acquired blood disorders characterized by ineffective hematopoiesis leading to cytopenias. Red blood cell transfusions are an important component of supportive care in patients with MDS. Prolonged exposure to transfusions can lead to iron overload, which results in iron-induced toxicity caused by the production of reactive oxygen species (ROS). ROS accumulation has detrimental effects also on hematopoietic stem cells and may contribute to MDS progression. The observation that iron chelation improves hematologic parameters and reduces transfusion dependence further indicates that iron overload impairs hematopoiesis. Over the past decade, the mechanisms regulating iron homeostasis and the complex interplay between iron overload and toxicity, ineffective hematopoiesis, and transformation to leukemia have become clearer. In this narrative review, we provide an overview of recent findings pertaining to iron overload in patients with MDS and its effects on hematopoiesis. We also briefly discuss the position of chelation therapy in the context of the new developments.
骨髓增生异常综合征（MDS）是一组克隆性获得性血液疾病，其特征为造血无效导致血细胞减少。红细胞输血是MDS患者支持性治疗的重要组成部分。长期输血可导致铁过载，进而由活性氧（ROS）生成引起铁诱导的毒性。ROS积累对造血干细胞也有有害影响，并可能促使MDS进展。铁螯合改善血液学参数并降低输血依赖性这一观察结果进一步表明铁过载损害造血功能。在过去十年中，调节铁稳态的机制以及铁过载与毒性、造血无效和白血病转化之间的复杂相互作用已变得更加清晰。在这篇叙述性综述中，我们概述了MDS患者铁过载的最新研究结果及其对造血的影响。我们还简要讨论了螯合疗法在新进展背景下的地位。
