小β地中海贫血患者与小β-α地中海贫血患者的血液指标比较

Bijan Keikhaei Dehdezi, Najmeh Namehgoshay Fard
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摘要

目的:地中海贫血症是最常见的遗传性疾病之一,是由于血红蛋白结构中的α和β球蛋白链的生成发生轻微紊乱而导致的一组遗传性血红蛋白疾病。目前还没有根据血液学结果区分地中海贫血类型的明确标准。在本研究中,我们旨在评估低度β地中海贫血(β-地中海贫血)指数与β-地中海贫血小儿和α地中海贫血(α-地中海贫血)的比较。方法和材料:在这项描述性分析研究中,共招募了 120 名患者,其中包括 80 名轻型β地中海贫血患者和 40 名轻型β地中海贫血合并α地中海贫血患者。所有患者均采集了 5cc 血液样本。测定红细胞参数,包括平均血球容积(MCV)、平均血球血红蛋白(MCH)、平均血球血红蛋白浓度(MCHC)和血红蛋白。MCV>80和A2>3.5为轻型地中海贫血,MCV<80和A2<3.5为重型地中海贫血或缺铁性贫血。结果显示结果显示,β地中海贫血组的血红蛋白、HCT、MCH、MCHC 和 MCV 平均值明显低于β地中海贫血伴α地中海贫血组(P <0.0001)。另一方面,对照组的这些指数水平明显高于两组患者(P<0.0001)。结果显示,β-α-地中海贫血组的血红蛋白 A2 百分比为 4.5 ± 0.91,明显高于β-地中海贫血组。与沉默型和特异性β+α地中海贫血组相比,β地中海贫血组的血红蛋白和MCV比率明显较低。此外,β地中海贫血组的血细胞比容率也明显低于特异性β地中海贫血组,但与沉默组无明显差异。结论根据我们的研究结果,尽管β-地中海贫血患者和β-α地中海贫血患者的某些血细胞比容指标存在差异,但这些指标不能作为鉴别指标。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Blood Indices of the Patients with β-Thalassemia Minor Compared to the Patients with β-Thalassemia Minor-Alpha-Thalassemia
Objective: Thalassemia, as one of the most common genetic diseases, is a group of hereditary hemoglobin disorders due to a slight disturbance in the production of alpha and beta globin chains in the structure of hemoglobin occurs. There are still no clear criteria for differentiating thalassemia types based on hematological findings. In the current study, we aimed to evaluate the low-grade beta-thalassemia (β-thalassemia) indices in comparison with β-thalassemia minor with alpha-thalassemia (α-thalassemia). Methods and materials: In this descriptive-analytic study, 120 patients were enrolled, including 80 patients with minor β-thalassemia and 40 patients with minor β-thalassemia with α-thalassemia. Of all patients, 5cc blood samples were taken. The red blood cell parameters including mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), and hemoglobin were determined. The level of MCV> 80 and A2> 3.5 β-thalassemia minor and MCV <80 and A2 <3.5 were considered as elevated thalassemia or iron deficiency anemia. Results: The results showed that the mean of hemoglobin, HCT, MCH, MCHC, and MCV in the β-thalassemia group was significantly lower than that of the β-thalassemia with α-thalassemia group (P <0.0001). On the other hand, the level of these indices in the control group was significantly higher than in the two groups of patients (P<0.0001). The results showed that the percentage of hemoglobin A2 in the β-alpha-thalassemia group was 4.5 ± 0.91, significantly higher than the β-thalassemia group. The rate of hemoglobin and MCV was significantly lower in the β-thalassemia group compared to the silent and trait β+αthalassemia group. Also, the rate of hematocrit was significantly lower in the β-thalassemia group compared to the trait, although had no significant difference with the silence group. Conclusion: Based on our findings, despite the difference between some hematocrit indices in the patients with β-thalassemia and β-αthalassemia, these indices cannot be used as differential indices.
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