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停用铁螯合疗法后,一名骨髓增生异常综合征/骨髓增殖性肿瘤重叠综合征患者实现持久的红细胞输注独立。

Durable Red Blood Cell Transfusion Independence in a Patient with an MDS/MPN Overlap Syndrome Following Discontinuation of Iron Chelation Therapy.

作者信息

Kochhar Harpreet, Leger Chantal S, Leitch Heather A

机构信息

Department of Medicine, University of St. Eustatius, Tucker, GA 30084, USA.

Division of Hematology, St. Paul's Hospital, University of British Columbia, Vancouver, BC, Canada V6Z 2A5.

出版信息

Case Rep Hematol. 2015;2015:253294. doi: 10.1155/2015/253294. Epub 2015 Mar 30.

Abstract

Background. Hematologic improvement (HI) occurs in some patients with acquired anemias and transfusional iron overload receiving iron chelation therapy (ICT) but there is little information on transfusion status after stopping chelation. Case Report. A patient with low IPSS risk RARS-T evolved to myelofibrosis developed a regular red blood cell (RBC) transfusion requirement. There was no response to a six-month course of study medication or to erythropoietin for three months. At 27 months of transfusion dependence, she started deferasirox and within 6 weeks became RBC transfusion independent, with the hemoglobin normalizing by 10 weeks of chelation. After 12 months of chelation, deferasirox was stopped; she remains RBC transfusion independent with a normal hemoglobin 17 months later. We report the patient's course in detail and review the literature on HI with chelation. Discussion. There are reports of transfusion independence with ICT, but that transfusion independence may be sustained long term after stopping chelation deserves emphasis. This observation suggests that reduction of iron overload may have a lasting favorable effect on bone marrow failure in at least some patients with acquired anemias.

摘要

背景。一些获得性贫血和输血性铁过载患者在接受铁螯合疗法(ICT)后出现血液学改善(HI),但关于停止螯合治疗后的输血状态信息较少。病例报告。一名低IPSS风险的RARS-T患者发展为骨髓纤维化,出现了定期红细胞(RBC)输血需求。对为期6个月的研究药物疗程或3个月的促红细胞生成素均无反应。在输血依赖27个月时,她开始服用地拉罗司,6周内实现了红细胞输血独立,螯合治疗10周时血红蛋白恢复正常。螯合治疗12个月后,停用了地拉罗司;17个月后,她仍保持红细胞输血独立,血红蛋白正常。我们详细报告了该患者的病程,并回顾了关于螯合治疗导致血液学改善的文献。讨论。有关于ICT实现输血独立的报道,但停止螯合治疗后输血独立可能长期维持这一点值得强调。这一观察结果表明,减少铁过载可能对至少一些获得性贫血患者的骨髓衰竭产生持久的有利影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d64a/4396885/d6e645acbbf9/CRIHEM2015-253294.001.jpg

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