肝豆状核变性（Wilson病）伴发Coombs试验阴性的急性血管内溶血十分罕见,近来本院收洽1例并抢救成功,现报道如下。1　资料 患者俞X,女性,18岁。因双上肢麻木、刺痛1周,头面部麻痛3d就诊,既往有慢性“肝炎”史,一直原因不明。门诊眼科发现其K-F环（+）,遂以“Wilson病”收治入院。入院体检无神经系统定位体征,无明显黄疸。实验室检查:血红细胞（RBC）4.05×10~（12）/L,白细胞（WBC）6.05×10~（9）/L,血红蛋白（Hb）127g/L,血小板（PLT）158×10~（9）/L;血丙氨酸氨基转移酶（ALT）32μ/L,天冬氨酸氨 Hepatolenticular degeneration (Wilson’s disease) associated with Coombs test-negative acute intravascular hemolysis is very rare, recently received a hospital contract and rescue success, are reported below. 1 data patient Yu X, female, 18 years old. Numbness due to upper extremities, sting for 1 week, head and face numbness 3d treatment, past history of chronic “hepatitis”, has been unknown. Outpatient ophthalmology found its K-F ring (+), then “Wilson disease” admitted to hospital. No physical examination admission signs of nervous system, no obvious jaundice. Laboratory tests showed that the RBC was 4.05 × 10-12 / W, WBC 6.05 × 10-9 / L, Hb 127g / L, PLT 158 × 10 ~ 9) / L; alanine aminotransferase (ALT) 32μ / L, aspartate ammonia