How we manage iron overload in sickle cell patients.
How we manage iron overload in sickle cell patients.
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DOI：
10.1111/bjh.14575
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发表时间：
2017-06
影响因子：
6.5
通讯作者：
Wood JC
中科院分区：
文献类型：
--
作者：
Coates TD;Wood JC
输血在镰状细胞病（SCD）患者的管理中起着重要作用，但会导致显著的铁过载。由于输血用于治疗SCD的严重并发症，因此仍然难以区分器官损伤是铁过载的结果还是由于输血治疗的并发症。更好的管理提高了生存率，但长期暴露于铁使SCD患者发生铁相关并发症的风险更大，应予以治疗。螯合疗法的成功取决于患者对处方治疗的依从性;因此，调整药物方案以增加对治疗的依从性至关重要。这篇综述将讨论目前SCD患者铁稳态的生物学，以及这如何告知我们的临床治疗方法。我们将介绍在我们中心通过磁共振成像对器官铁进行系列评估来治疗铁过载的临床方法。
Blood transfusion plays a prominent role in the management of patients with sickle cell disease (SCD), but causes significant iron overload. As transfusions are used to treat the severe complications of SCD, it remains difficult to distinguish whether organ damage is a consequence of iron overload or is due to the complications treated by transfusion. Better management has resulted in increased survival, but prolonged exposure to iron puts SCD patients at greater risk for iron-related complications that should be treated. The success of chelation therapy is dominated by patient adherence to prescribed treatment; thus, adjustment of drug regimens to increase adherence to treatment is critical. This review will discuss the current biology of iron homeostasis in patients with SCD and how this informs our clinical approach to treatment. We will present the clinical approach to treatment of iron overload at our centre using serial assessment of organ iron by magnetic resonance imaging.