Transplantation for Liver Failure in Patients With Sickle Cell Disease: Challenging But Feasible
Transplantation for Liver Failure in Patients With Sickle Cell Disease: Challenging But Feasible
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DOI：
10.1002/lt.22257
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发表时间：
2011-04-01
影响因子：
4.6
通讯作者：
Duvoux, Christophe
中科院分区：
文献类型：
--
作者：
Hurtova, Monika;Bachir, Dora;Duvoux, Christophe
镰状细胞病（SCD）经常影响肝脏;如果发生急性肝功能衰竭（ALF），唯一可能有效的治疗选择是肝移植（LT）。到目前为止，仅描述了12例因SCD相关ALF而进行LT的患者。我们报告了6例成人SCD患者（3男3女，中位年龄= 40.1岁）的回顾性系列研究，这些患者接受了紧急LT。LT的适应症是5例患者（3例丙型肝炎/铁过载诱导的肝硬化，2例铁过载诱导的肝硬化）的ALF合并肝硬化; 1例患者患有自身免疫性肝炎。中位随访时间为52.7个月（0.5-123个月）。1、3、5和10年生存率分别为83.3%、66.7%、44.4%和44.4%。1例患者在LT后第10天死于超急性同种异体移植排斥反应引起的肝细胞衰竭。LT后不久，2例患者发生癫痫发作;在1例病例中，癫痫发作是早期钙调神经磷酸酶诱导的白质脑病的并发症。4名长期存活者受益于SCD的特定管理;具体而言，血红蛋白S分数维持在30%以下，总血红蛋白水平维持在8 - 10 g/dL之间。2例患者出现轻度血管闭塞危象。3例患者出现丙型肝炎病毒（HCV）感染复发;其中2例患者在接受抗病毒治疗时出现可逆性神经系统并发症。精心挑选的SCD患者可能受益于紧急LT。然而，这些患者似乎特别容易发生LT后的神经系统并发症。相反，如果提供特定的管理，严重的SCD相关的危机似乎不会复发。如果可以最大限度地减少神经系统并发症，结果可能会得到改善;例如，可以推迟钙调磷酸酶抑制剂的给药，并可以改善对HCV感染复发的管理。肝移植17：381-392，2011。(C)2011年AASLD。
Sickle cell disease (SCD) frequently affects the liver; if acute liver failure (ALF) develops, the only potentially effective therapeutic option is liver transplantation (LT). Only 12 patients for whom LT was performed for SCD-related ALF have been described so far. We report a retrospective series of 6 adult patients with SCD (3 men and 3 women, median age = 40.1 years) who underwent emergency LT. The indication for LT was ALF complicating cirrhosis in 5 patients (hepatitis C/iron overload-induced cirrhosis in 3 and iron overload-induced cirrhosis in 2); one patient had autoimmune hepatitis. The median follow-up was 52.7 months (0.5-123 months). The 1-, 3-, 5-, and 10-year survival rates were 83.3%, 66.7%, 44.4%, and 44.4%, respectively. One patient died of hepatocellular failure precipitated by hyperacute allograft rejection on post-LT day 10. Soon after LT, 2 patients developed seizures; in 1 case, the seizures were a complication of early calcineurin inhibitor-induced leukoencephalopathy. Four long-term survivors benefited from specific management of SCD; specifically, the hemoglobin S fraction was maintained below 30% and the total hemoglobin level was maintained between 8 and 10 g/dL. Two patients had mild vaso-occlusive crises. Three patients experienced a recurrence of hepatitis C virus (HCV) infection; 2 of these patients experienced reversible neurological complications while they were receiving antiviral treatment. Carefully selected patients with SCD may benefit from emergency LT. However, such patients seem to be particularly susceptible to neurological complications after LT. In contrast, severe SCD-related crises do not seem to recur if specific management is provided. Outcomes may be improved if the neurological complications can be minimized; for example, the administration of a calcineurin inhibitor can be delayed, and the management of HCV infection recurrence can be improved. Liver Transpl 17:381-392, 2011. (C) 2011 AASLD.