Steinberg M H
VA Medical Center, Jackson, MS 39216.
Am J Med Sci. 1993 May;305(5):312-3. doi: 10.1097/00000441-199305000-00009.
Hemoglobin A2 levels in members of an African American family with -88 C-->T beta(+)-thalassemia were measured, and two patients in whom iron deficiency anemia developed were evaluated during treatment. Iron deficiency may diminish the level of HbA2 in healthy control subjects and in patients with heterozygous beta-thalassemia; in addition, it may reduce the amount of variant hemoglobin in certain hemoglobinopathies. Although iron deficiency appeared to be associated with a reduction in HbA2 quantity in the patient with heterozygous beta-thalassemia, the level of HbA2 did not fall below the range characteristic of beta-thalassemia. It had been proposed that mutations in the beta-globin gene promoter may be associated with higher-than-expected levels of HbA2. However, this "mild" beta-globin gene promoter mutation did not seem to result in HbA2 concentrations higher than anticipated in the heterozygous beta-thalassemias.
对一个患有 -88 C→T β(+)地中海贫血的非裔美国家庭成员的血红蛋白A2水平进行了测量，并对两名出现缺铁性贫血的患者在治疗期间进行了评估。缺铁可能会降低健康对照受试者和杂合子β地中海贫血患者的HbA2水平；此外，它可能会减少某些血红蛋白病中变异血红蛋白的量。虽然缺铁似乎与杂合子β地中海贫血患者HbA2量的减少有关，但HbA2水平并未降至β地中海贫血的特征范围以下。有人提出，β珠蛋白基因启动子的突变可能与高于预期的HbA2水平有关。然而，这种“轻度”的β珠蛋白基因启动子突变似乎并未导致杂合子β地中海贫血中HbA2浓度高于预期。
