Service d'Anatomie-Pathologique, Hôpital Beaujon AP-HP, Clichy and Institut National de la Santé et de la Recherche Médicale U773, Centre de Recherche Biomédicale Bichat Beaujon CRB3, Université Denis Diderot-Paris 7, France.
J Hepatol. 2011 Mar;54(3):455-61. doi: 10.1016/j.jhep.2010.07.038. Epub 2010 Oct 28.
BACKGROUND & AIMS: Previous studies on obliterative portal venopathy (OPV) have been biased due to the selection of patients with non-cirrhotic portal hypertension. The aim of this study was to clarify the characteristics of OVP diagnosed by liver biopsy.
Fifty-nine consecutive patients with OPV were retrospectively selected on strict histological criteria. Clinical, laboratory, portal vein patency, and associated disorders potentially involving vascular alterations were analyzed. The occurrence of complications was recorded during follow-up.
Mean age at diagnosis was 38.5±15 years old. Initial presentation was portal hypertension (64% of patients) and/or extrahepatic portal vein thrombosis (EHPVT) (22%) or isolated abnormal laboratory tests (20%). Associated diseases found at diagnosis were: prothrombotic disorders (30% of patients) and immune-mediated disorders (17%); 53% of patients had no causal factor (idiopathic OPV). During follow-up (median 8.6 years, range 1-23 years), features of portal hypertension worsened in 46% of patients; EHPVT and portal hypertension were finally found in 44% and 88% of patients. Anti-coagulation and beta-blockers were administered in 47% and 59% of patients, respectively. Severe complications (liver transplantation and/or death) occurred in 11 (19%) patients, 8 had idiopathic OPV. Patients with prothrombotic disorders received earlier anticoagulation therapy; all survived without transplantation.
A confident diagnosis of OPV can be done by biopsy and is conceivable in patients under 40 years without clinically significant portal hypertension. Poor outcome was noted in 19% of patients, most of them affected with idiopathic OPV. Patients with prothrombotic disorders received early anticoagulation and appeared to have a better outcome despite a high proportion of EHPVT.
既往关于闭塞性门静脉病（OPV）的研究存在偏倚，因为这些研究选择的患者是非肝硬化性门静脉高压。本研究旨在阐明经肝活检诊断的 OPV 的特征。
严格按照组织学标准，回顾性选择 59 例连续 OPV 患者。分析了临床、实验室、门静脉通畅性以及可能涉及血管改变的相关疾病。记录了随访期间并发症的发生情况。
诊断时的平均年龄为 38.5±15 岁。首发表现为门静脉高压（64%的患者）和/或肝外门静脉血栓形成（EHPVT）（22%）或孤立性异常实验室检查（20%）。诊断时发现的相关疾病有：血栓形成性疾病（30%的患者）和免疫介导性疾病（17%）；53%的患者无病因（特发性 OPV）。在中位随访 8.6 年（1-23 年）期间，46%的患者门静脉高压加重；最终有 44%和 88%的患者发现 EHPVT 和门静脉高压。分别有 47%和 59%的患者接受抗凝和β受体阻滞剂治疗。11 例（19%）患者发生严重并发症（肝移植和/或死亡），其中 8 例为特发性 OPV。有血栓形成性疾病的患者接受了更早的抗凝治疗；所有患者均存活且未进行移植。
通过肝活检可以明确诊断 OPV，且在无明显临床门静脉高压的 40 岁以下患者中可以明确诊断。19%的患者预后不良，其中大多数为特发性 OPV。有血栓形成性疾病的患者接受了早期抗凝治疗，尽管 EHPVT 比例较高，但预后似乎更好。
