Living related liver transplantation in an adult patient with hepatocellular adenoma and carcinoma 13 years after bone marrow transplantation for Fanconi anemia: A case report.
Living related liver transplantation in an adult patient with hepatocellular adenoma and carcinoma 13 years after bone marrow transplantation for Fanconi anemia: A case report.
复制标题
DOI：
10.1111/hepr.12043
复制
发表时间：
2013-09
期刊：
影响因子：
--
通讯作者：
Van Vlierberghe H
中科院分区：
文献类型：
--
作者：
Colle I;Laureys G;Raevens S;Libbrecht L;Leroy JG;Reyntjens K;Geerts A;Rogiers X;Troisi RI;Hoehn H;Schindler D;Hanenberg H;De Wilde V;Van Vlierberghe H
范可尼贫血是一种遗传性骨髓衰竭综合征，其特征是DNA修复失败。造血干细胞移植虽然可以治疗骨髓衰竭，但不能预防或治愈恶性肿瘤的发展等其他表现。我们描述了一位患有范可尼贫血和马凡氏综合征的26岁男性患者，他于1994年成功地接受了来自他hla相同的妹妹的干细胞骨髓移植。2006年，三例肝细胞癌（HCC）病变被发现并及时切除。切除标本包含3个病变，均显示β -catenin通路的激活：一个分化良好的脂肪性HCC伴其产生的腺瘤残余，一个腺瘤伴小灶分化良好的HCC和一个积胆性腺瘤。发生HCC的已知危险因素包括范可尼贫血本身和移植前3年使用雄激素（氧美洛酮）。由于发生其他HCC的风险增加，考虑到免疫抑制会增加其他恶性肿瘤的风险，建议进行肝移植。通过使用13年前已经作为骨髓供体的hla相同的姐妹的部分肝脏，可以避免免疫抑制。患者于2007年7月2日行左肝移植手术，供体和受体均无立即并发症。肝移植9个月后，受者出现吻合口胆道狭窄，必须经皮经肝胆道造影进行扩张。然而，两个月后，狭窄复发，需要手术再吻合（肝空肠吻合术）。肝移植5年后，病人的情况仍然很好。该病例报告具有双重意义，因为它是第一例报告与马凡氏综合征相关的范可尼贫血的病例，也是第一例报道的范可尼贫血通过活体同源hla供者的肝移植治疗肝细胞癌而不使用免疫抑制的病例。
Fanconi anemia is an inherited bone marrow failure syndrome, characterised by failing DNA repair. Hematopoetic stem cell transplantation, known to be curative for the bone marrow failure, does neither prevent or cure other manifestations such as the development of malignancies. We describe a 26-year-old male patient with known Fanconi anemia and Marfan syndrome who in 1994 underwent a successful bone marrow transplantation of stem cells from his HLA-identical sister. In 2006, three hepatocellular carcinoma (HCC) lesions in the liver were detected and promptly resected. The resection specimen contained 3 lesions, all showing activation of the beta-catenin pathway: a well differentiated steatotic HCC with remnants of the underlying adenoma from which it arose, an adenoma with small foci of well differentiated HCC and a cholestatic adenoma. Known risk factors for developing HCC include Fanconi anemia itself and the use of androgens (oxymetholone) for a period of 3 years preceeding transplantation. Because of the increased risk of developing additional HCC’s, liver transplantation was proposed, taking into account that immunosuppression increases the risk of other malignancies. By using part of the liver of the HLA-identical sister, already acting as bone marrow donor 13 years before, immunosuppression could be avoided. A left lobe liver transplantation was performed without immediate complications for donor and acceptor on July 2, 2007. Nine months after liver transplantation the recipient developed an anastomotic biliary stricture that had to be dilated by percutaneous transhepatic cholangiography. Two months later however, the stenosis recurred, necessitating a surgical reanastomosis (hepaticojejunostomy). Five years after liver transplantation the patient is still doing well. This case report is twofold special being the first case reporting Fanconi anemia linked to Marfan syndrome and being the first reported case of Fanconi anemia who was treated for hepatocellular carcinoma by liver transplantation from a living related HLA-identical donor without the use of immunosuppression.
登录
查看更多内容
影响因子：
3.5
作者：
LINARES, M;PASTOR, E;GRAU, E
通讯作者：
GRAU, E
影响因子：
30.8
作者：
Kim, Yonghwan;Lach, Francis P.;Desetty, Rohini;Hanenberg, Helmut;Auerbach, Arleen D.;Smogorzewska, Agata
通讯作者：
Smogorzewska, Agata
影响因子：
3.7
作者：
Hu Z;Zhao X;Li Y;Liu X;Zhang Q
通讯作者：
Zhang Q
影响因子：
6.2
作者：
Hoogduijn, Martin J.;Popp, Felix C.;Dahlke, Marc H.
通讯作者：
Dahlke, Marc H.
影响因子：
5
作者：
HOLDER, LE;GNARRA, DJ;PERKINS, P
通讯作者：
PERKINS, P