Jaber Rawshan Zuhair, Hassan Meàad Kadhum, Al-Salait Sadeq Khalaf
Center for Hereditary Blood Diseases, Basrah Heath Directorate, Basrah, Iraq.
Department of Pediatrics, College of Medicine, University of Basrah, Basrah, Iraq.
Blood Res. 2019 Mar;54(1):38-44. doi: 10.5045/br.2019.54.1.38. Epub 2019 Mar 21.
Microcytic anemia, the most common form of anemia in children and adolescents, is a heterogeneous group of diseases that is acquired or inherited. We assessed the frequency and causes of microcytosis in children and adolescents with the sickle cell trait (SCT).
This descriptive study included 95 subjects (49 males and 46 females) with SCT who attended Basra Center for Hereditary Blood Diseases for evaluation. Investigations included complete blood count, high performance liquid chromatography, capillary electrophoresis, and measurement of serum ferritin and transferrin levels.
SCT subjects had a low hemoglobin (Hb) concentration (9.79±1.75 g/dL), low mean corpuscular volume (MCV, 67.43±9.22), low mean corpuscular Hb (21.15±3.64), and a normal red cell distribution width (RDW, 14.00±2.30). Among 95 SCT subjects, 81 (85.26%) had microcytosis, 12 (12.63%) had normal MCV, and 2 (2.11%) exhibited macrocytosis. Sixty-three (77.78%) SCT subjects with microcytosis were iron deficient, and 18 (22.22%) had normal iron levels. The mean serum ferritin and HbA2 levels were significantly lower, while the RDW, sickle Hb, and serum transferrin levels were significantly higher in patients with microcytosis and iron deficiency compared to non-iron deficient subjects (<0.05). Correlation coefficients did not reveal a significant association between the MCV and iron status of SCT subjects (>0.05).
Despite the frequent occurrence of iron deficiency in SCT subjects, co-inheritance of alpha-thalassemia seemed to be the cause of low MCV in non-iron deficient individuals with microcytosis. Genetic analysis is required to understand the genetic basis of this phenomenon.
小细胞性贫血是儿童和青少年中最常见的贫血形式，是一组后天获得性或遗传性的异质性疾病。我们评估了患有镰状细胞性状（SCT）的儿童和青少年中红细胞平均容积减小的发生率及病因。
这项描述性研究纳入了95名到巴士拉遗传性血液病中心接受评估的患有SCT的受试者（49名男性和46名女性）。检查项目包括全血细胞计数、高效液相色谱法、毛细管电泳法以及血清铁蛋白和转铁蛋白水平的测定。
患有SCT的受试者血红蛋白（Hb）浓度较低（9.79±1.75 g/dL），平均红细胞体积（MCV，67.43±9.22）较低，平均红细胞血红蛋白含量（21.15±3.64）较低，红细胞分布宽度（RDW，14.00±2.30）正常。在95名患有SCT的受试者中，81名（85.26%）存在红细胞平均容积减小，12名（12.63%）的MCV正常，2名（2.11%）表现为红细胞平均容积增大。63名（77.78%）存在红细胞平均容积减小的患有SCT的受试者缺铁，18名（22.22%）铁水平正常。与非缺铁受试者相比，存在红细胞平均容积减小且缺铁的患者血清铁蛋白和HbA2水平显著降低，而RDW、镰状血红蛋白和血清转铁蛋白水平显著升高（<0.05）。相关系数未显示患有SCT的受试者的MCV与铁状态之间存在显著关联（>0.05）。
尽管患有SCT的受试者中缺铁情况频繁发生，但α地中海贫血的共同遗传似乎是红细胞平均容积减小的非缺铁个体中MCV降低的原因。需要进行基因分析以了解这一现象的遗传基础。
