Michel M, Lafaurie M, Noël V, Pico L, Bastie A, Godeau B, Schaeffer A
Service de médecine interne, hôpital Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil, France.
Rev Med Interne. 2001 Mar;22(3):280-3. doi: 10.1016/s0248-8663(00)00329-5.
The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hemolytic anemia is an unusual complication of Wilson's disease.
We report two cases of Wilson's disease revealed by acute intravascular hemolytic anemia associated with liver failure. Blood smear analysis showed stippled red cells in one case; hemolytic anemia improved within a few weeks in both patients but progressive liver failure required transplantation in the other. Hemolysis probably results from the toxic effect of free serum copper on erythrocyte membrane.
Diagnosis of Wilson's disease must be considered in case of acute hemolytic anemia associated with liver failure in young adults.
肝脏和中枢神经系统是威尔逊病（一种遗传性铜代谢紊乱疾病）的常见靶器官。严重溶血性贫血是威尔逊病的一种罕见并发症。
我们报告了两例因急性血管内溶血性贫血合并肝衰竭而确诊的威尔逊病病例。一例患者的血涂片分析显示有红细胞点彩；两名患者的溶血性贫血在几周内均有所改善，但另一例患者进行性肝衰竭需要进行肝移植。溶血可能是由于游离血清铜对红细胞膜的毒性作用所致。
对于年轻成人出现急性溶血性贫血合并肝衰竭的情况，必须考虑威尔逊病的诊断。
