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伊朗南部重型和中间型地中海贫血患者血清铁调素与铁蛋白水平的关系

Relationship Between Serum Hepcidin and Ferritin Levels in Patients With Thalassemia Major and Intermedia in Southern Iran.

作者信息

Haghpanah Sezaneh, Esmaeilzadeh Masoomeh, Honar Naser, Hassani Fatemeh, Dehbozorgian Javad, Rezaei Narges, Abdollahi Maryam, Bardestani Marzieh, Safaei Sanaz, Karimi Mehran

机构信息

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.

Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, IR Iran.

出版信息

Iran Red Crescent Med J. 2015 Jul 1;17(7):e28343. doi: 10.5812/ircmj.17(5)2015.28343. eCollection 2015 Jul.

Abstract

BACKGROUND

Hepcidin is a key regulator of iron absorption in humans. It is mainly affected by hypoxia and iron stores.

OBJECTIVES

The current study aimed to determine the correlation between serum hepcidin and ferritin levels in patients with Thalassemia Major (TM) and Thalassemia Intermedia (TI).

PATIENTS AND METHODS

The current cross-sectional study investigated 88 randomly selected patients with Thalassemia, 48 TM and 40 TI, registered at the Thalassemia Clinic of Shiraz University of Medical Sciences, a referral center for Thalassemia in Southern Iran in 2013. All patients with TI were receiving Hydroxyurea (HU) 10 - 15 mg/kg/day for at least 10 years. The serum hepcidin, ferritin levels, hemoglobin (Hb) and nucleated Red Blood Cell (RBC) of the two groups were measured.

RESULTS

No statistically significant correlation was observed between serum hepcidin and ferritin levels in any of the two groups of patients with TM (rs = 0.02, P = 0.892) or TI (rs = 0.055, P = 0.734). The median Interquartile Range (IQR) for serum hepcidin and ferritin levels were significantly higher in TM compared to TI group, (hepcidin: 87.6 (43.9) vs. 51.8 (23.4), P < 0.001; ferritin: 2208 (3761) vs. 465 (632), P < 0.001).

CONCLUSIONS

There was insignificant correlation between serum hepcidin and ferritin levels in the two groups of patients with TM and TI. It seems that regulation of hepcidin in patients with Thalassemia is more affected by erythropoeitic activity than iron stores. Also, hepcidin levels were significantly higher in patients with TM than TI, possibly due to higher erythropoeitic activity in TI. In TI, it seems that low dose HU increases Hb levels and leads to transfusion-independence, but it is not high enough to suppress bone marrow activity and ineffective erythropoiesis. Consequently, serum hepcidin level decreases.

摘要

背景

铁调素是人体铁吸收的关键调节因子。它主要受缺氧和铁储备的影响。

目的

本研究旨在确定重型地中海贫血(TM)和中间型地中海贫血(TI)患者血清铁调素与铁蛋白水平之间的相关性。

患者与方法

本横断面研究调查了2013年在伊朗南部地中海贫血转诊中心设拉子医科大学地中海贫血诊所登记的88例随机选择的地中海贫血患者,其中48例为TM,40例为TI。所有TI患者接受羟基脲(HU)10 - 15mg/kg/天治疗至少10年。测量两组患者的血清铁调素、铁蛋白水平、血红蛋白(Hb)和有核红细胞(RBC)。

结果

在TM组(rs = 0.02,P = 0.892)或TI组(rs = 0.055,P = 0.734)的任何一组患者中,血清铁调素与铁蛋白水平之间均未观察到统计学上的显著相关性。与TI组相比,TM组血清铁调素和铁蛋白水平的中位数四分位数间距(IQR)显著更高,(铁调素:87.6(43.9)对51.8(23.4),P < 0.001;铁蛋白:2208(3761)对465(632),P < 0.oo1)。

结论

TM和TI两组患者血清铁调素与铁蛋白水平之间的相关性不显著。地中海贫血患者中铁调素的调节似乎受红细胞生成活性的影响大于铁储备。此外,TM患者的铁调素水平显著高于TI患者,这可能是由于TI患者的红细胞生成活性较高。在TI患者中,低剂量HU似乎可提高Hb水平并导致无需输血,但不足以抑制骨髓活性和无效的红细胞生成。因此,血清铁调素水平降低。

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