肝豆状核变性临床表现复杂多样 ,当无神经系统症状时 ,容易误诊 ,尤其以溶血性贫血为首发或主要症状时。本文报道一家姐妹 2例均以溶血性贫血为首发症状。病例　患儿女 ,6岁 ,因全身黄染 10天 ,尿呈酱油色 7天入院。外院查血红蛋白 40ｇ/Ｌ ,网织红细胞 0 .0 35 ,诊断“溶血性 Hepatolenticular degeneration complicated and diverse clinical manifestations, when no neurological symptoms, easy to misdiagnosis, especially hemolytic anemia as the first or main symptoms. This article reports a sisters two cases are hemolytic anemia as the first symptom. Case of children, 6 years old, due to systemic yellow dye 10 days, urine was soy sauce color 7 days admission. Outside the hospital check hemoglobin 40g / L, reticulocytes 0. 0 35, the diagnosis of "hemolytic