Madan N, Sikka M, Sharma S, Rusia U
Department of Pathology, UCMS and GTB Hospital, Shahdara, Delhi, India.
Ann Hematol. 1998 Sep;77(3):93-6. doi: 10.1007/s002770050421.
Iron status was estimated in 463 heterozygous beta-thalassemics to delineate the effect of iron deficiency on the expression of hemoglobin A2 (HbA2) in these patients. One hundred and twenty-six (27.2%) patients with the trait were iron deficient. These iron-deficient patients had a significantly (p < 0.0002) higher prevalence of anemia (90.5%) compared with iron-replete patients with the trait (71.5%). The mean hemoglobin (Hb) concentration, MCV, and MCH were significantly (p < 0.0001) lower in patients with beta-thalassemia traits (BTT) who had iron deficiency than in those without iron deficiency. Mean RBC count and MCHC did not differ in the two groups. Mean HbA2 was not significantly different in the two groups of patients with the trait and was elevated (>3.5%) in all but one heterozygote investigated. Mean HbA2/cell was significantly (p < 0.05) lower in BTT patients with iron deficiency than in patients without iron deficiency. The presence of iron deficiency did not preclude the detection of BTT in this population. The effect of iron deficiency in BTT was apparent as a significant lowering of the Hb concentration and an increased prevalence of anemia. Iron therapy is warranted for BTT patients with iron-deficiency traits and would help to significantly raise their Hb concentration. The elevation of HbA2 was striking and could be used with reliability in making the diagnosis of BTT even in the presence of iron deficiency.
对463例杂合子β地中海贫血患者的铁状态进行评估，以明确缺铁对这些患者血红蛋白A2（HbA2）表达的影响。126例（27.2%）具有该特征的患者缺铁。与铁充足的具有该特征的患者相比，这些缺铁患者贫血患病率显著更高（90.5%）（p < 0.0002），后者为71.5%。缺铁的β地中海贫血特征（BTT）患者的平均血红蛋白（Hb）浓度、平均红细胞体积（MCV）和平均红细胞血红蛋白含量（MCH）显著低于（p < 0.0001）无缺铁的患者。两组的平均红细胞计数和平均红细胞血红蛋白浓度（MCHC）无差异。两组具有该特征的患者的平均HbA2无显著差异，且除1例受调查的杂合子外，所有患者的HbA2均升高（>3.5%）。缺铁的BTT患者的平均HbA2/细胞显著低于（p < 0.05）无缺铁的患者。缺铁并不妨碍在该人群中检测出BTT。缺铁对BTT的影响表现为Hb浓度显著降低和贫血患病率增加。对于具有缺铁特征的BTT患者，有必要进行铁治疗，这将有助于显著提高他们的Hb浓度。HbA2的升高很显著，即使在缺铁的情况下，也可可靠地用于诊断BTT。
