Lipid profile in β-thalassemia intermedia patients:: correlation with erythroid bone marrow activity
Lipid profile in β-thalassemia intermedia patients:: correlation with erythroid bone marrow activity
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DOI：
10.1111/j.1751-553x.2006.00862.x
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发表时间：
2007-06-01
影响因子：
3
通讯作者：
Di Concilio, R.
中科院分区：
文献类型：
--
作者：
Amendola, G.;Danise, P.;Di Concilio, R.
在所有表型的β-地中海贫血中，都有反复报道的脂质异常，包括血清中所有组份的低水平血脂。出乎意料的是，在最近的研究中，发现中间β地中海贫血(TI)患者的总胆固醇(TC)和高密度脂蛋白胆固醇(HDLC)和低密度脂蛋白胆固醇(LDL-C)浓度甚至低于重型地中海贫血患者，但对这些发现的病理生理没有明确的解释。这种信息的缺乏促使我们评估了我科追踪的TI患者的血脂和脂蛋白模式；这些数据与遗传性球形红细胞增多症患者的数据进行了比较。此外，在两组患者中，利用血浆中可溶性转铁蛋白受体(STfR)的水平来评估红系骨髓活性。两组患者均表现出相似的血脂异常(低TC、低密度脂蛋白胆固醇和低密度脂蛋白胆固醇)和相同的sTfR升高，但TI患者的血红蛋白水平显著降低。我们研究的数据分析表明，TI患者的血脂谱不受年龄、性别、肝损伤、血红蛋白或铁蛋白水平的影响；红系骨髓活性增高和胆固醇消耗增加可能是TI患者血脂异常的主要机制。
Lipid abnormalities, including low levels of all fractions of serum lipids, have been repeatedly reported in all phenotypes of beta-thalassemia. Unexpectedly, in more recent studies, the concentration of total cholesterol (TC) and high- and/or low-density lipoprotein cholesterol (HDL-C and LDL-C) has been found in beta-thalassemia intermedia (TI) patients even lower than in thalassemia major, without a clear explanation of pathophysiology of these findings. This lack of information prompted us to evaluate the plasma lipids and lipoproteins pattern in the TI patients followed in our department; the data were compared with those found in hereditary spherocytosis patients. Furthermore, in both groups of patients, the erythroid bone marrow activity was evaluated, utilizing the level of soluble transferrin receptors (sTfR) in the plasma. Both groups of patients showed similar lipid abnormalities (low-TC, HDL-C and LDL-C) and the same increase of sTfR, with significantly lower hemoglobin levels in TI patients. Data analysis of our study shows that the lipid profile in TI patients is not influenced by age, sex, liver injury, hemoglobin or ferritin levels; the higher erythroid bone marrow activity with the enhanced cholesterol consumption could be the dominant mechanism implicated in the lipid abnormalities of TI patients.