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骨髓中广泛的铁过载:一名重型地中海贫血患者全血细胞减少的原因——病例报告

Extensive iron overload in bone marrow: A cause of pancytopenia in a thalassemia major patient - A case report.

作者信息

Ali Maria, Ali Sidra Asad, Mansoori Huma

机构信息

Section of Haematology, Department of Pathology, Jinnah Medical College, Karachi, Pakistan.

Section of Haamatology, Laboratory, Patel Hospital, Karachi, Pakistan.

出版信息

Asian J Transfus Sci. 2020 Jul-Dec;14(2):195-197. doi: 10.4103/ajts.AJTS_11_18. Epub 2020 Dec 19.

Abstract

Iron overload-associated organ damage in transfusion-dependent anemias is a well-known phenomenon. Here, we discuss a case of 28-year-old, poorly chelated thalassemia major patient, whose blood workup revealed pancytopenia and moderately raised serum ferritin levels. His bone marrow examination was performed which revealed massive iron overload. Aggressive iron chelation led to successful recovery of peripheral blood counts in his patient. This case focuses on the importance of early detection and timely management of reversible iron overload toxicities. Serum ferritin although is convenient marker to asses iron overload, but it should not be relied upon to assess the severity of iron overload. Hence, organ-specific diagnostic modalities must be used along with serum ferritin to assess the severity of iron overload to prevent long-term complications in patients with regular blood transfusions.

摘要

输血依赖型贫血中与铁过载相关的器官损伤是一种众所周知的现象。在此,我们讨论一例28岁、螯合治疗不佳的重型地中海贫血患者,其血液检查显示全血细胞减少和血清铁蛋白水平中度升高。对其进行了骨髓检查,结果显示存在大量铁过载。积极的铁螯合治疗使该患者的外周血细胞计数成功恢复。本病例强调了早期发现和及时处理可逆性铁过载毒性的重要性。血清铁蛋白虽是评估铁过载的便捷指标,但不应仅依赖它来评估铁过载的严重程度。因此,必须结合血清铁蛋白使用器官特异性诊断方法来评估铁过载的严重程度,以预防长期输血患者出现长期并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/565b/7983133/e798c7984904/AJTS-14-195-g001.jpg

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