Dover G J, Boyer S H, Zinkham W H, Kazazian H H, Pinney D J, Sigler A
Blood. 1977 Mar;49(3):355-65.
Fetal and adult erythrocyte characteristics were studied serially in a 30-mo-old female with juvenile chronic myelocytic leukemia. On presentation the erythrocytes exhibited predominantly fetal characteristics as indicated by 69% hemoglobin F (HbF), 1.1% hemoglobin A2 (HbA2), absent I antigen, and fetal levels of the erythrocyte enzymes, carbonic anhydrase I and II, glucose-6-phosphate dehydrogenase, hexokinase, pyruvate kinase, and lactate dehydrogenase; 100% of the erythrocytes present contained HbF. However, Orskov-Jacobs-Stewart hemolysis demonstrated that at least one adult characteristic was present. Seven months later HbF was 17%; I antigen and carbonic anhydrase I had increased to adult levels. The number of cells containing HbF had decreased to 30%. Further studies indicated that at least three new populations of red cells were present after 7 mo which had not previously been detected. Two of these populations exhibited a mixture of both fetal and adult characteristics. Such findings suggested that an ongoing disturbance of regulatory mechanisms was responsible for the variable expression of fetal versus adult erythrocyte characteristics.
对一名30个月大患青少年慢性粒细胞白血病的女性患者的胎儿和成人红细胞特征进行了连续研究。初诊时，红细胞主要表现出胎儿特征，血红蛋白F（HbF）为69%，血红蛋白A2（HbA2）为1.1%，I抗原缺失，红细胞酶碳酸酐酶I和II、葡萄糖-6-磷酸脱氢酶、己糖激酶、丙酮酸激酶和乳酸脱氢酶水平为胎儿水平；所有存在的红细胞中100%含有HbF。然而，奥斯科夫-雅各布斯-斯图尔特溶血试验表明至少存在一种成人特征。7个月后，HbF为17%；I抗原和碳酸酐酶I已升至成人水平。含HbF的细胞数量降至30%。进一步研究表明，7个月后至少出现了三个先前未检测到的新红细胞群体。其中两个群体表现出胎儿和成人特征的混合。这些发现表明，调节机制的持续紊乱是胎儿与成人红细胞特征可变表达的原因。
