骨髓增生性肿瘤引起的巴德-恰里综合征的肝移植--管理和长期效果。

IF 0.8 4区医学 Q4 IMMUNOLOGY

Transplantation proceedings Pub Date : 2024-09-05 DOI:10.1016/j.transproceed.2024.08.040

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引用次数: 0

摘要

骨髓增生性肿瘤可引起原发性巴德-恰里综合征，并伴有急性或慢性肝功能衰竭，必须进行肝移植。然而，预防复发仍具有挑战性，移植后抗凝和细胞再生治疗的必要性也不够明确。我们分析了 2004 年至 2021 年期间到我科就诊的所有 MPN PBCS 患者的治疗方案。八名患者接受了肝移植手术，其中六名是由于急性肝功能衰竭。移植后，所有患者都接受了抗凝治疗，7 名患者接受了细胞修复治疗。移植后的平均存活时间为 13.25 年。肝移植与移植后抗凝治疗和细胞再生治疗相结合，可获得良好的长期疗效。

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Liver Transplantation for Budd-Chiari Syndrome From Myeloproliferative Neoplasms - Management and Long-Term Results

Myeloproliferative neoplasms can cause primary Budd-Chiari-Syndrome with acute or chronic liver failure necessitating liver transplantation. However, preventing the recurrence remains challenging and the need for post-transplant anticoagulant and cytoreductive treatment is not sufficiently clear. We analyzed the treatment regimens for all patients who presented to our department with PBCS from MPN between 2004 and 2021. Eight patients underwent liver transplantation - 6 of them due to an acute liver failure. Post-transplant, all patients received anticoagulant and 7 patients cytoreductive medication. The mean survival after transplantation was 13.25 years. Liver transplantation shows favorable long-term outcome when combined with post-transplant anticoagulant and cytoreductive treatment.

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来源期刊

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.