Correction of anemia in a transfusion-dependent patient with primary myelofibrosis receiving iron chelation therapy with deferasirox (Exjade®, ICL670)
Correction of anemia in a transfusion-dependent patient with primary myelofibrosis receiving iron chelation therapy with deferasirox (Exjade®, ICL670)
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DOI：
10.1111/j.1600-0609.2007.00840.x
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发表时间：
2007-06-01
影响因子：
3.1
通讯作者：
Angelucci, Emanuele
中科院分区：
文献类型：
--
作者：
Di Tucci, Anna Angela;Murru, Roberta;Angelucci, Emanuele
慢性贫血患者的输血铁超负荷可导致多器官功能衰竭。骨髓增生异常综合征患者铁超载的管理经验有限，因为许多患者由于预期寿命短以及与当前参考标准螯合剂去铁胺给药相关的困难而不接受螯合治疗。然而，有一些报告称，骨髓增生异常综合征和骨髓纤维化患者的螯合治疗可减少输血需求。在这里，我们讨论了一个病人与原发性骨髓纤维化和相关的输血依赖性贫血谁接受螯合治疗与每日一次口服铁螯合剂，地拉罗司。除了铁水平降低外，患者还表现出输血需求的意外减少，最终导致长期无输血生存。
Transfusional iron overload in patients with chronic anemias can result in multiple organ failure. Experience in the management of iron overload in patients with myelodysplastic syndromes is limited, as many do not receive chelation therapy due to short-life expectancy and the difficulties associated with the administration of the current reference standard chelator, deferoxamine. There have, however, been some reports of reduced transfusion requirement associated with chelation therapy in patients with myelodysplastic syndromes and myelofibrosis. Here, we discuss a patient with primary myelofibrosis and related transfusion-dependent anemia who received chelation therapy with the once-daily oral iron chelator, deferasirox. In addition to the reduced iron levels, the patient demonstrated an unexpected reduction in blood transfusion requirement, ultimately resulting in long-lasting transfusion-free survival.