Lorenzo Olivero , Hong Liang , Ian A. Makey , Si M. Pham , Jorge Sinclair , Stephen Aniskevich III , Sadia Z. Shah , Dana K. Perry , Wesley L. Allen , Nathan H. Waldron , Liu Yang , Pramod K. Guru , Candido E. Rivera , Pablo Moreno Franco , Tathagat Narula
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引用次数: 0
摘要
肺肝联合移植(CLLT)是一种罕见的治疗终末期肺病和肝病的干预措施。对于因血友病 A 和肝凝血功能障碍而导致出血风险增加的患者来说,这是一项挑战。我们介绍了首例有记录的 CLLT 病例,患者是一名患有血友病 A、丙型肝炎病毒相关性肝硬化和特发性肺纤维化(IPF)的老年男性。尽管患者的肝功能和血友病病情稳定,但他的肺部状况却迅速恶化,因此被列入移植名单。患者成功接受了 CLLT,多学科团队协调了围手术期管理,以应对血友病 A 的独特挑战,从而在术中纠正了凝血病。患者术后恢复良好,无需进行因子 FVIII 替代治疗。本病例表明,CLLT 可以治疗三种病理生理不同的疾病:终末期肺病和肝病,并纠正 A 型血友病表型。我们的报告为有关 CLLT 是否适用于 A 型血友病患者的有限文献做出了贡献。
Combined lung and liver transplant for cirrhosis, idiopathic pulmonary fibrosis, and hemophilia A: Case report
Combined lung and liver transplantation (CLLT) is a rare intervention for end-stage lung and liver diseases. It poses a challenge for patients with increased bleeding risk due to Hemophilia A and liver coagulopathy. We present the first documented case of CLLT in an elderly male with Hemophilia A, HCV-associated cirrhosis, and Idiopathic Pulmonary Fibrosis (IPF). Despite the patient exhibiting stable liver function and hemophilia, his lung condition rapidly deteriorated, prompting the listing for transplant. The patient underwent a successful CLLT with perioperative management coordinated by the multidisciplinary team to address the unique challenges of Hemophilia A, resulting in intra-operative correction of coagulopathy. The patient exhibited a favorable recovery, with no requirement for Factor FVIII replacement therapy postoperatively. This case demonstrates that CLLT can address three diseases with distinct pathophysiologies: end-stage lung and liver disease, and correct the hemophilia A phenotype. Our report contributes to the limited literature on the suitability of CLLT in patients with hemophilia A.
期刊介绍:
To provide to national and regional audiences experiences unique to them or confirming of broader concepts originating in large controlled trials. All aspects of organ, tissue and cell transplantation clinically and experimentally. Transplantation Reports will provide in-depth representation of emerging preclinical, impactful and clinical experiences. -Original basic or clinical science articles that represent initial limited experiences as preliminary reports. -Clinical trials of therapies previously well documented in large trials but now tested in limited, special, ethnic or clinically unique patient populations. -Case studies that confirm prior reports but have occurred in patients displaying unique clinical characteristics such as ethnicities or rarely associated co-morbidities. Transplantation Reports offers these benefits: -Fast and fair peer review -Rapid, article-based publication -Unrivalled visibility and exposure for your research -Immediate, free and permanent access to your paper on Science Direct -Immediately citable using the article DOI