Zaizov R, Steinherz M, Wollach B, Kirschmann C
Acta Haematol. 1980;64(3):136-40. doi: 10.1159/000207226.
Hematological data and globin synthesis studies in 8 alpha-thalassemic children whose parents are from various regions in the Middle East are reported. All patients were devoid of Hb H. 5 of them had mild anemia, hypochromia and microcytosis and their blood alpha-/non-alpha-globin radioactivity ratios ranged between 0.56 and 0.75. The other 3 patients were hematologically normal with blood radioactivity ratios between 0.77 and 0.88. Bone marrow studies showed balanced globin chain synthesis for 6 of the patients and relatively more alpha-globin synthesis than in the blood for the remaining 2. The decrease in the relative synthesis of alpha-globin on erythroid cell maturation may relate to the molecular basis of alpha-thalassemia in the Middle East.
本文报道了8名父母来自中东不同地区的α地中海贫血患儿的血液学数据和珠蛋白合成研究。所有患者均无Hb H。其中5例有轻度贫血、低色素血症和小红细胞症，其血液α/非α珠蛋白放射性比值在0.56至0.75之间。另外3例血液学检查正常，血液放射性比值在0.77至0.88之间。骨髓研究显示，6例患者珠蛋白链合成平衡，其余2例α珠蛋白合成相对血液中更多。红系细胞成熟时α珠蛋白相对合成量的减少可能与中东地区α地中海贫血的分子基础有关。
