Successful Living Donor Liver Transplantation from a Donor With Low von Willebrand Factor Activity: A Case Report

IF 0.8 4区 医学 Q4 IMMUNOLOGY
Takahiko Omameuda, Taiichi Wakiya, Yasunaru Sakuma, Yasuharu Onishi, Yukihiro Sanada, Noriki Okada, Yuta Hirata, Toshio Horiuchi, Kiichiro Takadera, Ryosuke Akimoto, Hironori Yamaguchi
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Abstract

Liver transplantation (LT) from donors with von Willebrand factor (VWF) abnormalities is rarely reported, largely due to concerns over donor safety and potential hemostatic complications in the recipient. Low VWF activity is more prevalent than von Willebrand disease and may present without bleeding symptoms, yet its implications in the context of living donor LT (LDLT) remain poorly characterized. We report a case of successful LDLT from a donor with borderline low VWF activity, highlighting detailed perioperative and long-term coagulation profiles in both donor and recipient. The donor, a healthy young woman with no personal or family history of bleeding, was found to have mildly decreased ristocetin cofactor activity. A test infusion of VWF/FVIII concentrate elicited a favorable biological response. Based on these findings and in accordance with clinical guidelines, open right hepatectomy was performed under perioperative VWF replacement. The donor experienced no bleeding or thrombotic complications and remained clinically stable during 18 months of follow-up. VWF-related parameters remained elevated postoperatively and ultimately returned to baseline or higher, indicating preserved long-term hemostasis. The recipient, who received the graft from the low-VWF donor, showed stable VWF-related coagulation parameters post-transplantation. Despite a transient decline following reperfusion, levels normalized within the expected timeframe and remained within normal range throughout follow-up. This case supports the safety of LDLT from donors with low VWF levels when appropriate perioperative management is applied. It also highlights that low donor VWF activity may not compromise recipient hemostasis, offering important implications for donor eligibility assessment in clinical practice.
低血管性血友病因子活性供者活体肝移植成功一例报告。
血管性血友病因子(VWF)异常供体的肝移植(LT)很少报道,主要是由于对供体安全性和受体潜在止血并发症的担忧。低VWF活性比血管性血友病更普遍,可能没有出血症状,但其在活体供体肝移植(LDLT)中的意义仍不清楚。我们报告一例来自边缘性低VWF活性供体的LDLT成功病例,强调了供体和受体的详细围手术期和长期凝血特征。供体为健康年轻女性,无个人或家族出血史,发现瑞斯托素辅助因子活性轻度降低。试验输注VWF/FVIII浓缩物引起了良好的生物学反应。根据这些发现,并按照临床指南,在围手术期VWF置换下进行了开放式右肝切除术。供体没有出血或血栓并发症,在18个月的随访中保持临床稳定。vwf相关参数术后保持升高,最终恢复到基线或更高,表明长期止血。接受低vwf供体移植的受者在移植后显示稳定的vwf相关凝血参数。尽管再灌注后出现短暂性下降,但在预期时间内水平恢复正常,并在随访期间保持在正常范围内。本病例支持低VWF供体在围手术期适当管理时LDLT的安全性。它还强调,低供体VWF活性可能不会影响受体止血,这为临床实践中供体资格评估提供了重要意义。
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来源期刊
Transplantation proceedings
Transplantation proceedings 医学-免疫学
CiteScore
1.70
自引率
0.00%
发文量
502
审稿时长
60 days
期刊介绍: Transplantation Proceedings publishes several different categories of manuscripts, all of which undergo extensive peer review by recognized authorities in the field prior to their acceptance for publication. The first type of manuscripts consists of sets of papers providing an in-depth expression of the current state of the art in various rapidly developing components of world transplantation biology and medicine. These manuscripts emanate from congresses of the affiliated transplantation societies, from Symposia sponsored by the Societies, as well as special Conferences and Workshops covering related topics. Transplantation Proceedings also publishes several special sections including publication of Clinical Transplantation Proceedings, being rapid original contributions of preclinical and clinical experiences. These manuscripts undergo review by members of the Editorial Board. Original basic or clinical science articles, clinical trials and case studies can be submitted to the journal?s open access companion title Transplantation Reports.
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