The etiology of chronic splanchnic vein thrombosis in adults: a two-center analysis.

American journal of blood research Pub Date : 2025-02-15 eCollection Date: 2025-01-01 DOI:10.62347/NMIJ8301
Esin Oguz Kozan, Gizem Isguzar, Enver Ucbilek, Serkan Yaras, Emel Gurkan, Orhan Sezgin, Mehmet Ali Sungur, Anil Tombak
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引用次数: 0

Abstract

Portal vein thrombosis (PVT) and Budd-Chiari syndrome (BCS) are rare vascular disorders with both well-recognized and less commonly identified etiologies.

Objectives: This study aims to investigate the etiologies of portal vein thrombosis (PVT) and Budd-Chiari syndrome (BCS), thereby enhancing improving early detection and management strategies for these conditions. A retrospective review was undertaken to identify the etiologies of PVT and BCS.

Methods: A detailed clinical evaluation was performed and all underlying diseases, such as MPD, and related conditions (e.g. surgery) associated with thrombosis were recorded.

Results: The study comprised a total of 73 patients, with 58 diagnosed with PVT and 15 with BCS. Of these patients, 56 (76.7%) had at least one underlying disease. The most prevalent underlying diseases in patients with PVT were cirrhosis (32/58, 55.2%), myeloproliferative disease (3/58, 5.2%), malignancy (4/58, 6.9%), and rheumatological conditions (4/58, 6.9%). For BCS, 11/15 patients (73.3%) had at least one predisposing condition, including cirrhosis in six cases. Congenital causes were identified in 16/58 cases of PVT (27.6%), in 7/15 cases of BCS (46.7%). Thirty-two patients had previously undergone gastrointestinal surgery (PVT 24/58, BCS 8/15); surgery was the sole etiology in 15/73 patients (20.5%). Homocysteinemia was common (PVT 20/58, BCS 5/15). A multitude of rare etiologies were identified, including paroxysmal nocturnal haemoglobinuria, Crohn's disease, nephrotic syndrome, drug therapies, pregnancy, JAK2 mutation, and elevated factor VIII or fibrinogen.

Conclusions: The presence of a wide range of diverse frequent-infrequent etiologies of congenital or acquired splanchnic vein thrombosis in this cohort underscores the necessity for the implementation of appropriate diagnostic strategies in a broad spectrum of at-risk patients.

成人慢性内脏静脉血栓形成的病因:一项双中心分析。
门静脉血栓形成(PVT)和Budd-Chiari综合征(BCS)是一种罕见的血管性疾病,其病因已得到很好的认识,但不太常见。目的:本研究旨在探讨门静脉血栓形成(PVT)和Budd-Chiari综合征(BCS)的病因,从而提高对这些疾病的早期发现和治疗策略。对PVT和BCS的病因进行回顾性分析。方法:进行详细的临床评估,并记录所有潜在疾病,如MPD,以及与血栓相关的相关情况(如手术)。结果:该研究共纳入73例患者,其中58例诊断为PVT, 15例诊断为BCS。在这些患者中,56例(76.7%)至少有一种基础疾病。PVT患者最常见的基础疾病是肝硬化(32/58,55.2%)、骨髓增生性疾病(3/58,5.2%)、恶性肿瘤(4/58,6.9%)和风湿病(4/58,6.9%)。对于BCS, 11/15(73.3%)患者至少有一种易感条件,包括6例肝硬化。先天性病因占PVT的16/58(27.6%),占BCS的7/15(46.7%)。32例患者既往行胃肠手术(PVT 24/58, BCS 8/15);手术是73例患者中15例(20.5%)的唯一病因。同型半胱氨酸血症很常见(PVT 20/58, BCS 5/15)。发现了多种罕见病因,包括突发性夜间血红蛋白尿、克罗恩病、肾病综合征、药物治疗、妊娠、JAK2突变和因子VIII或纤维蛋白原升高。结论:在本队列中,先天性或获得性内脏静脉血栓形成的各种常见或罕见病因的存在强调了在广泛的高危患者中实施适当诊断策略的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
American journal of blood research
American journal of blood research MEDICINE, RESEARCH & EXPERIMENTAL-
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