Treatment of recurrent allograft dysfunction with intravenous hematin after liver transplantation for erythropoietic protoporphyria
Treatment of recurrent allograft dysfunction with intravenous hematin after liver transplantation for erythropoietic protoporphyria
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DOI：
10.1097/00007890-200203270-00014
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发表时间：
2002-03-27
期刊：
影响因子：
6.2
通讯作者：
Chung, RT
中科院分区：
文献类型：
--
作者：
Dellon, ES;Szczepiorkowski, ZM;Chung, RT
红细胞生成性原卟啉(EPP)是一种罕见的遗传性血红素生物合成途径障碍，在这种疾病中，毒性水平的原卟啉经常在肝脏中沉淀，导致肝硬化、肝功能衰竭和需要肝移植(OLT)。由于EPP中潜在的酶缺陷来自骨髓，因此EPP同种异体移植功能障碍复发的风险很高。虽然血浆置换可以改善急性同种异体移植物疾病，但维持疾病缓解的策略是必要的。一位59岁的男性患者接受了肝EPP的原位肝移植，术后第700天胆红素和转氨酶升高。同种异体移植物活检显示EPP复发。他最初的治疗方法是血浆置换、过度输血和静脉注射。血红素。在肝功能测试正常化后，血红素输注是间歇性的，耐受性良好，并与正常的同种异体移植功能相关近2年。这是第一例在原位肝移植后使用血红素来帮助实现和维持同种异体移植物EPP疾病的缓解。
Erythropoietic protoporphyria (EPP) is a rare inherited disorder of the heme biosynthetic pathway in which toxic levels of protoporphyrins often precipitate in the liver, leading to cirrhosis, liver failure, and the need for liver transplantation (OLT). Because the underlying enzyme defect in EPP is bone marrow derived, the risk for recurrent EPP allograft dysfunction is high. Although plasmapheresis may ameliorate acute allograft disease, strategies to maintain disease remission are needed. A 59-year-old man who underwent OLT for hepatic EPP experienced increased bilirubin and aminotransferases on postoperative day 700. Allograft biopsy demonstrated recurrent EPP. He was managed initially with plasmapheresis, hypertransfusion, and infusions of i.v. hematin. After normalization of liver tests, the hematin infusions have been given intermittently, are well tolerated, and associated with normal allograft function for nearly 2 years. This is the first case of the use of hematin given post-OLT to help achieve and maintain remission of allograft EPP disease.