Kiss J E, Berman D, Van Thiel D
Division of Hematology/Bone Marrow Transplantation, University of Pittsburgh School of Medicine, Pennsylvania, USA.
Transfusion. 1998 Apr;38(4):327-31. doi: 10.1046/j.1537-2995.1998.38498257369.x.
Patients who present with fulminant hepatic failure due to Wilson's disease may develop hemolytic anemia and renal insufficiency. In this entity, acute hepatocellular necrosis triggers the release of copper ions into the circulation, which leads to toxic effects on red cell metabolic pathways and hemolysis.
The utility of therapeutic plasma exchange to rapidly remove copper and reduce toxic serum copper levels was studied in two patients with fulminant Wilson's disease.
Intensive plasma exchange using fresh-frozen plasma replacement removed substantial amounts of copper from the hypercupremic patients, resulting in a rapid reduction in serum copper levels and decreased hemolysis. The net copper removal was proportional to the serum level, ranging from 7,000 to 11,800 micrograms per procedure in one patient and from 3,700 to 6,800 micrograms in the other.
Plasma exchange allows a rapid reduction in elevated serum copper levels in patients with fulminant Wilson's disease. This leads to an amelioration of hemolytic anemia and provides clinical stabilization until liver transplantation can be performed.
因威尔逊病出现暴发性肝衰竭的患者可能会发生溶血性贫血和肾功能不全。在此类病症中，急性肝细胞坏死促使铜离子释放进入循环系统，进而对红细胞代谢途径产生毒性作用并导致溶血。
对两名暴发性威尔逊病患者研究了治疗性血浆置换在快速清除铜并降低血清铜毒性水平方面的效用。
使用新鲜冷冻血浆置换进行强化血浆置换，从高铜血症患者体内清除了大量铜，使血清铜水平迅速降低且溶血减轻。净铜清除量与血清水平成正比，一名患者每次操作清除7000至11800微克，另一名患者每次操作清除3700至6800微克。
血浆置换可使暴发性威尔逊病患者升高的血清铜水平迅速降低。这会改善溶血性贫血并在能够进行肝移植之前实现临床稳定。
