Portal Thrombosis in Cirrhosis: Role of Thrombophilic Disorders.

José Ignacio Fortea,Ana Batlle López,Emilio Fábrega,Marcos López Hoyos,Ángela Puente,Paloma Álvarez Fernández,Javier Nuñez Céspedes,Antonio Cuadrado,Francisco José González Sanchez,Patricia Huelin,Javier Crespo,María Del Rocío Pérez Montes,Carmen Álvarez Tato,Inés García Carrera

doi:10.3390/jcm9092822

Abstract

In patients with liver cirrhosis the contribution of inherited and acquired prothrombotic disorders in the development of non-malignant portal vein thrombosis (PVT) is inconclusive. The purpose of this retrospective study was to examine the prevalence of thrombophilia in this setting at our center from January 2012 to November 2019. Tests included gene mutational analysis for Factor V Leiden, prothrombin G20210A, JAK2 (V617F), Calreticulin (CARL), in addition to activated protein C resistance, antithrombin III, protein C and S levels, and antiphospholipid antibodies. We included 77 patients, six of whom (7.8%) had a thrombophilic disorder: antiphospholipid syndrome in four patients, prothrombin gene mutation in one and factor V Leiden mutation in one. This latter patient had also been diagnosed with polycythemia vera years before PVT development. Complete thrombosis of the main portal vein and re-thrombosis after stopping anticoagulation were more frequent in patients with thrombophilia, but the rates of recanalization under anticoagulant therapy were similar among groups. No other difference was accounted between groups. The low prevalence of acquired and inherited thrombophilia found in patients with cirrhosis and PVT support testing for these disorders on an individual basis and avoiding universal screening to reduce costs and unwarranted testing.

Highlights

Non-malignant portal vein thrombosis (PVT) is defined as a thrombus that develops within the portal vein trunk and intrahepatic portal branches, which may involve the splenic (SV)
The latter transformation can develop very early after acute PVT, it is generally used to define the chronic stage of PVT [1]
Discrepancies among studies regarding patient selection criteria, degree and extent of thrombosis, treatment strategies, sample size and time of follow-up have led to conflicting data [6]

Summary

Introduction

Non-malignant portal vein thrombosis (PVT) is defined as a thrombus that develops within the portal vein trunk and intrahepatic portal branches, which may involve the splenic (SV)or superior mesenteric veins (SMV). In the absence of recanalization, the portal venous lumen is obliterated and portoportal collaterals develop resulting in portal cavernoma. The latter transformation can develop very early after acute PVT, it is generally used to define the chronic stage of PVT [1]. It constitutes the most common thrombotic event in patients with cirrhosis, with increased rates in the setting of advanced liver disease. The reported prevalence of PVT varies with different diagnostic methods and target populations, ranging between approximately 10–25% in patients with decompensated cirrhosis and 1–5% in those with compensated cirrhosis [2]. There is no consensus on its optimal management and no definitive recommendations have been reported in clinical guidelines or consensus conferences [1,4,5,7,8]

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