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1960年Scott等氏发现一种致家族性Heinz小体贫血症的异常血红蛋白。以后,各国报道类似的异常血红蛋白计有7种,由于这些血红蛋白具有自动解离或聚合,容易氧化为高铁血红蛋白,或者对热不稳定等特性,Huehns和Shooter氏称之为不稳定血红蛋白。本文报道在上海发现的患遗传性不稳定血红蛋白病的一个家系。根据患者异常血红蛋白的电泳特性、不稳定性、吸收光谱和临床特征
In 1960 Scott et al. Found an abnormal hemoglobin that caused familial Heinz’s body anemia. Since then, there have been seven reports of similar abnormal hemoglobin in various countries. Huehns and Shooter called them unstable hemoglobin because of their auto-dissociation or polymerization, their susceptibility to methemoglobin or their thermal instability. This article reports a pedigree of hereditary unstable hemoglobin disease found in Shanghai. According to patients with abnormal hemoglobin electrophoresis characteristics, instability, absorption spectra and clinical features