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. 2019 Sep 10;3(4):658-667.
doi: 10.1002/rth2.12253. eCollection 2019 Oct.

Inherited thrombophilia and portal vein thrombosis in cirrhosis: A systematic review and meta-analysis

Steven D Ma  1 , Jennifer Wang  2 , Dmitri Bezinover  3 , Zakiyah Kadry  4 , Patrick G Northup  5 , Jonathan G Stine  6   7
Affiliations

Inherited thrombophilia and portal vein thrombosis in cirrhosis: A systematic review and meta-analysis

Steven D Ma et al. Res Pract Thromb Haemost. .

Abstract

Background: Portal vein thrombosis (PVT) is common in cirrhosis. PVT is associated with high morbidity and mortality. Individual reports suggest that PVT occurs more frequently in patients with cirrhosis and inherited thrombophilia. The relationship between cirrhosis, PVT development, and inherited thrombophilia was explored in this study. The aim of the study was to determine whether cirrhotic patients with nontumoral PVT have an increased rate of inherited thrombophilia.

Methods: Studies were identified by searching electronic databases up to October 2017 with English language and human subject restrictions. Two independent reviewers screened citations and extracted data. Magnitude of effect was calculated to obtain aggregate estimates of effect size and 95% confidence intervals (CIs). Between-study variability and heterogeneity were assessed.

Results: Of 2893 citations identified, 9 studies composed of 1929 subjects with cirrhosis were included. The overall prevalence of PVT was 6.5% (n = 125). Both prothrombin G20210A mutation (odds ratio [OR], 2.43; 95% CI, 1.07-5.53; P = 0.03) and factor V Leiden (FVL) (OR, 1.98; 95% CI, 1.06-3.68; P = 0.03) were significantly associated with PVT risk. Methyltetrahydrofolate reductase C677T mutation was not associated with increased PVT risk. No heterogeneity or publication bias was observed. One important study with opposite findings could not be included due to lack of primary data.

Conclusions: FVL and PTG20210A mutation were associated with increased PVT risk in patients with cirrhosis. This finding reframes the role of inherited thrombophilia in PVT development in patients with cirrhosis. Future prospective studies investigating screening for inherited thrombophilia in all cirrhosis patients with PVT seem warranted.

Keywords: factor V Leiden; hereditary; mutation; portal vein thrombosis; prothrombin; thrombophilia.

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Figures

Figure 1
Figure 1
Flow chart of study inclusion
Figure 2
Figure 2
Pooled measure of effects for patients with cirrhosis with/without PVT and with/without presence of: A, Factor V Leiden (FVL); B, prothrombin mutation (PTG20210A); C, methyltetrahydrofolate reductase mutation (MTHFR C677T)
Figure 3
Figure 3
Funnel plot analyzing publication bias for: A, factor V Leiden (FVL); B, prothrombin mutation (prothrombin G20210A); C, methyltetrahydrofolate reductase mutation (MTHFR C677T)
See this image and copyright information in PMC

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