Knapp A B, Crawford J M, Rappeport J M, Gollan J L
Gastroenterology. 1987 Feb;92(2):513-9. doi: 10.1016/0016-5085(87)90150-8.
A 28-yr-old woman with severe idiopathic aplastic anemia received an HLA-identical mixed lymphocyte culture nonreactive bone marrow transplant from her brother. In the months after successful engraftment, she developed cutaneous and hepatic graft-versus-host disease, associated with marked cholestatic jaundice. Despite a series of therapeutic maneuvers, cholestasis persisted but remained relatively stable over the ensuing 10 yr. However, serial liver biopsies revealed progressive biliary-type fibrosis culminating in cirrhosis. Subsequently, her clinical course deteriorated and she developed signs of hepatic failure, and ultimately died 10.5 yr after bone marrow transplantation. The evolution of chronic graft-versus-host disease to cirrhosis may be a limiting factor in the long-term survival of this group of bone marrow transplant recipients. The lack of correlation between the stable clinical or biochemical indices and the progressive hepatic disease underscores the need for sequential liver biopsies in patients with sustained liver function abnormalities after bone marrow transplantation.
一名28岁的严重特发性再生障碍性贫血女性接受了来自其兄弟的HLA配型相合且混合淋巴细胞培养无反应的骨髓移植。在成功植入后的数月里,她出现了皮肤和肝脏移植物抗宿主病,并伴有明显的胆汁淤积性黄疸。尽管采取了一系列治疗措施,胆汁淤积仍持续存在,但在随后的10年里保持相对稳定。然而,系列肝脏活检显示进行性胆管型纤维化最终发展为肝硬化。随后,她的临床病程恶化,出现肝功能衰竭的体征,最终在骨髓移植后10.5年死亡。慢性移植物抗宿主病演变为肝硬化可能是这组骨髓移植受者长期生存的一个限制因素。稳定的临床或生化指标与进行性肝脏疾病之间缺乏相关性,突出了对骨髓移植后肝功能持续异常患者进行系列肝脏活检的必要性。