肝豆状核变性是较常见的常染色体隐性遗传性铜代谢障碍性疾病，临床多表现为锥体外系症状、肝病症状、角膜色素环（ＫＦ环）。少数病人以溶血性贫血为首发症状，临床常易误诊。现将我们近２０年来收治的６例以溶血性贫血为首发症状的肝豆状核变性病例报告如下。临床资料一、 Hepatolenticular degeneration is a common autosomal recessive inherited copper metabolism disorders, clinical manifestations of extrapyramidal symptoms, symptoms of liver disease, corneal pigment ring (K  F ring). A small number of patients with hemolytic anemia as the first symptom, often misdiagnosed clinically. Now we nearly 20 years admitted to 6 cases of hemolytic anemia as the first symptom of Wilson’s disease reported as follows. First,