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中间型地中海贫血中的血清铁蛋白

Serum ferritin in thalassemia intermedia.

作者信息

Shah Ravi, Trehan Amita, Das Reena, Marwaha R K

机构信息

Department of Pediatrics, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.

Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.

出版信息

Indian J Hematol Blood Transfus. 2014 Dec;30(4):281-5. doi: 10.1007/s12288-013-0267-y. Epub 2013 Jun 6.

Abstract

Serum ferritin is a useful monitoring tool for iron overload in thalassemia major. In resource poor settings access to modalities for assessment of iron overload are limited. This study was undertaken to assess the efficiency and usefulness of serum ferritin level in thalassemia intermedia (TI) patients. This was a cross sectional observational study. Seventy seven TI patients attending the pediatric hematology clinic were included. Fasting blood sample was taken from each patient in iron free vials for iron studies. Serum ferritin was estimated by immunometric enzyme immunoassay using Orgentec GmbH kits. Mean age of patients evaluated was 10.9 ± 5.03 (3-26) years. The mean age at diagnosis was 4.21 ± 2.3 (0.8-11) years. Mean serum ferritin was 486.54 ± 640.0 ng/ml (15-4,554). Thirty two (41.5 %) patients had a ferritin value of ≥500 ng/ml. Nine patients had a serum ferritin of ≥1,000 ng/ml. Three of the subjects with a ferritin >1,000 ng/ml had never received a blood transfusion (BT) and in the other six, the number of BTs ranged from 1 to 8. Serum ferritin did not correlate with age, total number of BTs splenectomy status or BT in last one year (p > 0.05). In 41.5 % of TI patients, serum ferritin was ≥500 ng/ml. Age, BT and splenectomized status did not affect ferritin level. We postulate interplay of other biological factors like HFE gene mutation, ferroportin, etc. to contribute to ferritin level and hence iron load in TI patients. Ferritin can possibly be used as screening and monitoring tool for iron load in TI patients when other modalities to assess iron overload are not easily available.

摘要

血清铁蛋白是监测重型地中海贫血铁过载的一种有用工具。在资源匮乏地区,评估铁过载的方式有限。本研究旨在评估血清铁蛋白水平在地中海贫血中间型(TI)患者中的有效性和实用性。这是一项横断面观察性研究。纳入了77名到儿科血液科门诊就诊的TI患者。在无铁试管中采集每位患者的空腹血样用于铁相关检查。使用德国奥根泰克有限公司试剂盒通过免疫酶免疫测定法估算血清铁蛋白。评估的患者平均年龄为10.9±5.03(3 - 26)岁。诊断时的平均年龄为4.21±2.3(0.8 - 11)岁。平均血清铁蛋白为486.54±640.0 ng/ml(15 - 4554)。32名(41.5%)患者的铁蛋白值≥500 ng/ml。9名患者的血清铁蛋白≥1000 ng/ml。铁蛋白>1000 ng/ml的受试者中有3名从未接受过输血(BT),另外6名患者的输血量在1至8次之间。血清铁蛋白与年龄、输血总数、脾切除状态或过去一年的输血量均无相关性(p>0.05)。在41.5%的TI患者中,血清铁蛋白≥500 ng/ml。年龄、输血情况和脾切除状态均不影响铁蛋白水平。我们推测其他生物因素如HFE基因突变、铁转运蛋白等之间的相互作用会影响TI患者的铁蛋白水平及铁负荷。当评估铁过载的其他方式不易获得时,铁蛋白可能可用作TI患者铁负荷的筛查和监测工具。

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本文引用的文献

1
Iron overload cardiomyopathy in clinical practice.
Circulation. 2011 Nov 15;124(20):2253-63. doi: 10.1161/CIRCULATIONAHA.111.050773.
2
Optimal management of β thalassaemia intermedia.
Br J Haematol. 2011 Mar;152(5):512-23. doi: 10.1111/j.1365-2141.2010.08486.x. Epub 2011 Jan 20.
4
Iron overload in thalassaemia intermedia: reassessment of iron chelation strategies.
Br J Haematol. 2009 Dec;147(5):634-40. doi: 10.1111/j.1365-2141.2009.07848.x. Epub 2009 Aug 13.
9
Secondary iron overload.
Hematology Am Soc Hematol Educ Program. 2001:47-61. doi: 10.1182/asheducation-2001.1.47.
10
Membrane-bound iron contributes to oxidative damage of beta-thalassaemia intermedia erythrocytes.
Br J Haematol. 2001 Jan;112(1):48-50. doi: 10.1046/j.1365-2141.2001.02482.x.

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