Melis M A, Rosatelli C, Falchi A M, Angius A, Furbetta M, Galanello R, Cao A
Acta Haematol. 1980;63(1):32-6. doi: 10.1159/000207365.
88 adults with thalassemia-like red cell indices, normal serum iron and normal hemoglobin (Hb) A2 and F levels, diagnosed in a mass screening had Hb H inclusion bodies studies (65 subjects) or Hb H inclusion bodies studies and globin chain synthesis analysis (23 subjects). The alpha/beta ratio of 0.70 +/- 0.10 was the same as in obligate alpha-thalassemia-1 (alpha-thal) carriers. Hb H inclusion bodies studies were found to be a reliable test for alpha-thal trait identification, resulting positive in approximately 70% of suspected carriers. The alpha-thal carrier defined by Hb H preparation or by globin chain synthesis had significant reduction in the mean Hb level, hematocrit, mean corpuscular hemoglobin and a significant increase in mean red cell count, but there was some overlap with controls.
在一次大规模筛查中被诊断出的88名具有地中海贫血样红细胞指数、血清铁正常且血红蛋白(Hb)A2和F水平正常的成年人,进行了Hb H包涵体研究(65名受试者)或Hb H包涵体研究及珠蛋白链合成分析(23名受试者)。0.70±0.10的α/β比值与纯合子α地中海贫血-1(α-地贫)携带者相同。发现Hb H包涵体研究是鉴定α-地贫特征的可靠检测方法,约70%的疑似携带者检测结果呈阳性。通过Hb H制备或珠蛋白链合成定义的α-地贫携带者,其平均Hb水平、血细胞比容、平均红细胞血红蛋白显著降低,平均红细胞计数显著增加,但与对照组存在一定重叠。