Pagnier J, Labie D
Biochimie. 1975;57(1):71-6. doi: 10.1016/s0300-9084(75)80111-8.
Hemoglobin chain synthesis during leukemic processes has been studied on patients having fetal hemoglobin. All cases showed the following abnormalities : (1) a relatively increased synthesis of the beta chain ; (2) an important increase of the free dimeric precursors pool, with, most of the time, a predominance of alpha chain. If the first point suggests an alpha-thalassemia feature, the presence of free alpha chains shows evidence for a more complex mechanism not only due to a decrease of messenger RNA. The hypothesis of a clonal disorder could neither be demonstrated nor ruled out. The observed abnormalities could be due to a defect in a alpha chain depending regulation mechanism.
对患有胎儿血红蛋白的白血病患者的血红蛋白链合成情况进行了研究。所有病例均显示出以下异常情况:(1)β链合成相对增加;(2)游离二聚体前体池显著增加,大多数情况下α链占优势。如果第一点提示有α地中海贫血特征,那么游离α链的存在表明存在一种更为复杂的机制,而不仅仅是由于信使核糖核酸减少所致。克隆性疾病的假说既无法得到证实,也无法排除。观察到的异常情况可能是由于α链依赖调节机制存在缺陷。