Neuberger J, Portmann B, Macdougall B R, Calne R Y, Williams R
N Engl J Med. 1982 Jan 7;306(1):1-4. doi: 10.1056/NEJM198201073060101.
Three patients who had undergone orthrotopic liver transplantation for primary biliary cirrhosis and were being maintained on immunosuppressive therapy were investigated 31/2 to 41/2 years later because of the redevelopment of pruritus and mild jaundice. In one patient pigmentation was again evident, and all three had a rise in the titer of serum mitochondrial antibody after an initial fall. Liver histology showed features of primary biliary cirrhosis with non-suppurative destructive cholangitis, lymphoid aggregates, and increased deposition of copper-binding protein in the absence of cholestasis. None of these features was found in patients who had received grafts for other conditions and had lived for comparable periods, nor were they found in patients who had had rejection with bile-duct abnormalities. The overall findings indicate a recurrence of primary biliary cirrhosis in the donor organ.
三名因原发性胆汁性肝硬化接受原位肝移植并接受免疫抑制治疗的患者,在3年半至4年半后因瘙痒和轻度黄疸复发而接受调查。其中一名患者色素沉着再次明显,所有三名患者血清线粒体抗体滴度在最初下降后又有所上升。肝脏组织学显示原发性胆汁性肝硬化的特征,伴有非化脓性破坏性胆管炎、淋巴样聚集物,且在无胆汁淤积的情况下铜结合蛋白沉积增加。在因其他疾病接受移植且存活时间相当的患者中未发现这些特征,在发生排斥反应并伴有胆管异常的患者中也未发现。总体研究结果表明供体器官中原发性胆汁性肝硬化复发。