Organ transplantation in hereditary apolipoprotein Al amyloidosis
Organ transplantation in hereditary apolipoprotein Al amyloidosis
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DOI：
10.1111/j.1600-6143.2006.01507.x
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发表时间：
2006-10-01
影响因子：
8.8
通讯作者：
Hawkins, P. N.
中科院分区：
文献类型：
--
作者：
Gillmore, J. D.;Stangou, A. J.;Hawkins, P. N.
遗传性载脂蛋白AI（apoAI）淀粉样变性患者通常有广泛的内脏淀粉样沉积，许多人在年轻时发展为终末期肾衰竭。由于该疾病的多系统性和进行性以及移植物中淀粉样蛋白复发的风险，用实体器官移植来替代系统性淀粉样变性中衰竭的器官功能存在争议。我们报告了10例apoAI淀粉样变性患者的实体器官移植结局，包括4例双移植，这些患者从诊断淀粉样变性和移植后分别随访了16（4-28）年和9（0.2-27）年。10例患者中有8例存活，7例在审查时移植物功能正常。两名患者死亡，一名患者在肾移植后2个月死于播散性巨细胞病毒感染，另一名患者在肾移植后13年多死于严重创伤后多系统衰竭。1例患者肾移植失败，25年后淀粉样蛋白复发。淀粉样蛋白疾病进展非常缓慢，在两例肝脏移植病例中，病情的自然史得到了有利的改变。遗传性apoAI淀粉样变性中出现衰竭的器官应该被替换，因为移植物存活率很高，并能带来显着的生存益处。
Patients with hereditary apolipoprotein AI (apoAI) amyloidosis often have extensive visceral amyloid deposits, and many develop end-stage renal failure as young adults. Solid organ transplantation to replace failing organ function in systemic amyloidosis is controversial due to the multisystem and progressive nature of the disease and the risk of recurrence of amyloid in the graft. We report the outcome of solid organ transplantation, including dual transplants in 4 cases, among 10 patients with apoAI amyloidosis who were followed for a median (range) of 16 (4-28) and 9 (0.2-27) years from diagnosis of amyloidosis and transplantation, respectively. Eight of 10 patients were alive, seven with a functioning graft at censor. Two patients died, one of disseminated cytomegalovirus infection 2 months after renal transplantation and the other of multisystem failure following severe trauma more than 13 years after renal transplantation. The renal transplant of one patient failed due to recurrence of amyloid after 25 years. Amyloid disease progression was very slow and the natural history of the condition was favorably altered in both cases in which the liver was transplanted. Failing organs in hereditary apoAI amyloidosis should be replaced since graft survival is excellent and confers substantial survival benefit.