Beris P, Boreux G, Klein D, Miescher P A
Nouv Rev Fr Hematol (1978). 1980;22(3):223-34.
A statistical analysis was done on the levels of Hb A2 and Hb F in 1 340 normal subjects and 356 subjects heterozygous for beta-thalassemia. This study revealed that both groups had a normal distribution of Hb A2 and a gaussologarithmic distribution of Hb F. The plotted curves show an overlapping zone between the two populations. This area lies between 3.0% and 3.5% Hb A2 and between 0.2% and 1.0% Hb F. More than 97% of the heterozygotes for beta-thalassemia have a Hb A2 level situated outside the overlapping region, which permits diagnosis. Of the remaining 3%, only the "silent carriers" do not manifest the usual hematological characteristics of beta-thalassemia minor (hypochromia, microcytosis, diminished osmotic fragility, and erythrocytes with basophilic stippling). To diagnose this group it is necessary to determine globulin chain synthesis. This test is reserved for those subjects considered as "high risks."
对1340名正常受试者和356名β地中海贫血杂合子受试者的Hb A2和Hb F水平进行了统计分析。该研究表明，两组的Hb A2均呈正态分布，Hb F呈高斯对数分布。绘制的曲线显示了两个人群之间的重叠区域。该区域位于Hb A2的3.0%至3.5%之间以及Hb F的0.2%至1.0%之间。超过97%的β地中海贫血杂合子的Hb A2水平位于重叠区域之外，这有助于诊断。在其余3%中，只有“沉默携带者”没有表现出轻型β地中海贫血的常见血液学特征（低色素、小红细胞症、渗透脆性降低以及嗜碱性点彩红细胞）。要诊断这一组，有必要测定球蛋白链合成。该检测仅适用于那些被视为“高风险”的受试者。
