论文部分内容阅读
来院诊治的2位女青年系姐妹,自幼患中度贫血症。血红蛋白定量表明:抗碱血红蛋白及HbA_2明显升高,HbA显著降低,诊断为胎儿血红蛋白的持续存在兼β地中海贫血。该症在我国尚未见报道,特提出报告,引起讨论。为了确证患者的抗碱Hb系胎儿Hb(HbF),作了Hb杂交及解链电泳。对照结果,患者抗碱Hb电泳区带与脐带血HbF迁移位置相同;解链电泳比较:脐带血HbF解链后的α、γ链的迁移位置与抗碱Hb两条肽链的位置相同。杂交实验也显示同样的结果。从而进一步证明患者的抗碱血红蛋白与脐带血HbF的一致性。本文对Clegg等人所报道的HPFH兼β地中海贫血的研究作了初步的比较讨论,也对HPFH的研究概况,如结构分析、遗传学研究等方面作了探讨。
Two young women who came to the hospital were sisters and had mild anemia at an early age. Quantitative analysis of hemoglobin showed that the anti-alkali hemoglobin and HbA 2 were significantly increased, HbA was significantly reduced, and the persistence of fetal hemoglobin and β-thalassemia were diagnosed. The disease has not been reported in our country, special report, aroused discussion. In order to confirm the patient’s anti-alkali Hb fetus Hb (HbF), made Hb hybridization and melting electrophoresis. The control group showed the same migration position of HbF in anti-alkali Hb electrophoresis zone and umbilical cord blood. The results of melting-chain electrophoresis showed that the migration positions of α and γ chains after cord blood HbF melting were the same as those of HbF. Hybridization experiments also showed the same result. Thus further demonstrating the patient’s anti-alkali hemoglobin and cord blood HbF consistency. In this paper, Clegg et al. Reported a preliminary comparative study of HPFH and β-thalassemia and also discussed the general situation of HPFH such as structural analysis and genetics research.