Alter B P, He L, Acosta R, Knobloch M E, Thomson J C, Giardina P, Weinberg R S
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029.
Hemoglobin. 1992;16(6):447-67. doi: 10.3109/03630269208993114.
Blood erythroid progenitors (BFU-E) from patients with sickle and thalassemic syndromes were compared with those from normal individuals. The day of maximal colony formation in methyl cellulose was slightly later in the cultures from the patients with hemoglobinopathies than in the normal cultures. The number of colonies/100,000 mononuclear cells was similar in all cultures on day 13, but was higher in the hemoglobinopathy cultures on the day of maximal growth. The number of BFU-E/mL of blood was significantly higher than normal at all times in both sickle cell anemia and thalassemia. The proportional synthesis of gamma globin was twice normal in all sickle cultures, and 4 times normal in those from beta+-thalassemia. Hemin and interleukin-3 increased the numbers of erythroid colonies in all cultures, but did not consistently alter the globin synthesis patterns. Each progenitor population has a unique pattern in terms of time course, number of BFU-E, and level of gamma globin synthesis. These features indicate distinct types of BFU-E, or differences in accessory cells, or both, which distinguish blood-borne erythropoiesis in normals and those with hemoglobinopathies.
将镰状细胞综合征和地中海贫血综合征患者的血液红系祖细胞（BFU-E）与正常个体的进行了比较。在甲基纤维素中形成集落最多的那天，血红蛋白病患者培养物中的时间比正常培养物稍晚。在第13天，所有培养物中每100,000个单核细胞的集落数相似，但在生长达到最大值那天，血红蛋白病培养物中的集落数更高。在镰状细胞贫血和地中海贫血中，血液中每毫升BFU-E的数量在所有时间都显著高于正常水平。在所有镰状细胞培养物中，γ珠蛋白的比例合成是正常的两倍，在β+地中海贫血患者的培养物中是正常的四倍。血红素和白细胞介素-3增加了所有培养物中红系集落的数量，但并未持续改变珠蛋白合成模式。就时间进程、BFU-E数量和γ珠蛋白合成水平而言，每个祖细胞群体都有独特的模式。这些特征表明存在不同类型的BFU-E，或辅助细胞存在差异，或两者皆有，这区分了正常人和血红蛋白病患者的血源性红细胞生成。
