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作者分析一个有慢性溶血性贫血的中国人患者的家系,采用醋酸纤维薄膜电泳和淀粉凝胶电泳法(pH8.6)检测结果发现患者不存在HbA,而出现异常Hb Leiden(电泳迁移率在HbA与HbA_2之间),利用CMC柱层分析和指纹法分析证明,患者的β链第6位或第7位谷氨酸残基缺如,按通式把异常Hb写为Hb Leiden(α_2β_2 6或7glu→0)。患者的父亲和哥哥呈现不稳定Hb Leiden的症状,父亲含Hb Leiden
The authors analyzed the pedigree of a Chinese patient with chronic hemolytic anemia using an acetate acetate thin film electrophoresis and a starch gel electrophoresis (pH 8.6) test that found no HbA and no abnormal Hb Leiden (electrophoretic mobility at HbA And HbA_2), CMC column analysis and fingerprinting analysis showed that the patient had missing glutamic acid residues at the 6th or 7th β-chain, according to the general formula Hb Leiden (α_2β_2 6 or 7glu → 0). The patient’s father and brother presented with symptoms of unstable Hb Leiden, his father containing Hb Leiden