Inherited coagulation disorders in cirrhotic patients with portal vein thrombosis

Amitrano, Lucio; Brancaccio, Vincenzo; Guardascione, Maria Anna; Margaglione, Maurizio; Iannaccone, Luigi; D’Andrea, Giovanna; Marmo, Riccardo; Ames, P. R. J.; Balzano, A.

doi:10.1002/hep.510310213

Cited by 252 publications

(193 citation statements)

References 38 publications

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“…9 PAI-1 4G-4G has come out as the leader factor in the SVT group, and in the various subgroups, confirming its important role in pathogenesis of SVT, as Balta et al 10 have found, and confirming itself as a thrombotic risk factor in different target organs, as showed by Tsantes et al 11 MTHFR 677TT genotype was strongly associated with portal thrombosis in our cirrhotic patients as reported previously by Amitrano et al 12 Hardy-Weinberg equilibrium for PAI-1 and MTHFR 677 was deviated from that expected from a population (PAI-1 x 2 41.96, P < 0.0001; MTHFR 677 x 2 15.00, P 0.0001). We think that PAI-1 4G-4G and MTHFR 677TT can increase the inflammation response, participating to the activation of perisinusoidal hepatic stellate cells, causing hepatic fibrosis and augmented intrahepatic vascular resistance typical of the LC, as suggested by Fernandez.…”

Section: Discussionsupporting

confidence: 86%

PAI-1 4G-4G, MTHFR 677TT, V Leiden 506Q, and Prothrombin 20210A in Splanchnic Vein Thrombosis: Analysis of Individual Patient Data From Three Prospective Studies

Pasta¹,

Pasta

D’Amico

2016

Journal of Clinical and Experimental Hepatology

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Background: There are no univocal opinions on the role of genetic thrombophilia on splanchnic vein thrombosis (SVT). We defined genetic thrombophilia the presence of one of these thrombophilic genetic factors (THRGFs): PAI-1 4G-4G, MTHFR 677TT, V Leiden 506Q, and prothrombin 20210A. Objectives: To evaluate the frequencies of these THRGFs in SVT patients, we analyzed individual data of 482 Caucasian patients, recruited from 2000 to 2014 in three prospective studies. SVT was defined as the presence of thrombosis of portal (PVT), mesenteric (MVT), splenic (SPVT), cava (CT), and hepatic vein (Budd Chiari syndrome, BCS). Pre-hepatic SVT (pre-HSVT) was defined as PVT with or without MVT/SPVT, without BCS. Post-hepatic SVT (post-HSVT) was BCS with or without PVT/MVT/SPVT. Methods: We compared 350 patients with liver cirrhosis (LC), 47 hepatocellular carcinoma (HCC), 37 myeloproliferative neoplasm (MPN), 38 associated disease (AD), 10 without any associated disease (WAD), vs 150 healthy controls (HC); 437 patients showed pre-HSVT and 45 post-HSVT. Results: Thrombophilia was present in 294/482 (60.9%) patients: 189/350 LC (54.0%), 31/47 (66.0%) HCC, 29/39 (74.4%) MPN, 35/38 AD (92.1%), and 10/10 (100%) WAD, and 54/150 (36.0%) in HC. In the total group, we found 175 PAI-1 4G-4G, 130 MTHFR 677TT, 42V Leiden 506Q, and 27 prothrombin 20210A; 75 patients showed presence of >1 TRHGF; the more frequent association was PAI-1 4G-4G/MTHFR 677TT, in 36 patients. PAI-1 4G-4G and MTHFR 677TT were significantly more frequent in patients with SVT (P values <0.005), whereas V Leiden Q506 and prothrombin G20210A were not. PAI-1 4G-4G and MTHFR 677TT distributions deviated significantly from that expected from a population in Hardy-Weinberg equilibrium. Thrombophilia was significantly less frequent in patients with pre-HSVT (250/437, 57.2%) than in patients with post-HSVT (44/45, 97.8%). Conclusions: Our study shows the significant prevalence of PAI-1 4G-4G and MTHFR 677TT in SVT, mainly in post-HSVT. ( J CLIN EXP HEPATOL 2016;6:10-14)

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Section: Discussionsupporting

confidence: 86%

PAI-1 4G-4G, MTHFR 677TT, V Leiden 506Q, and Prothrombin 20210A in Splanchnic Vein Thrombosis: Analysis of Individual Patient Data From Three Prospective Studies

Pasta¹,

Pasta

D’Amico

2016

Journal of Clinical and Experimental Hepatology

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“…A recent meta-analysis questioned the utility of anti-thrombin, protein C, and protein S levels to predict PVT [34], while measurement of congenital deficiencies of anticoagulants may be informative in patients with cirrhosis and a personal or familial history of thrombosis. Carriers of polymorphisms of factor V or prothrombin gene were at increased risk of PVT [35], as were those with increased levels of factor VIII [36]. Anti-phospholipid syndrome also carries a risk for thrombosis in the population without cirrhosis; however, the laboratory diagnosis of this syndrome which is based on phospholipid-dependent coagulation tests, is unreliable in patients with cirrhosis, who have abnormal coagulation tests at baseline.…”

Section: Testing For Thrombophiliamentioning

confidence: 99%

“…PVT is frequent in cirrhosis [35,48,[179][180][181][182][183][184][185][186][187][188][189][190], with a 1-year incidence of 4.6-16.7% (Table 4), and prevalence of 1.4-26.1% (Table 5). Prevalence increases with the severity of cirrhosis [186,190], with rates of up to 44.4% in patients with hepatocellular carcinoma.…”

Section: Portal Vein Thrombosis In Cirrhosismentioning

confidence: 99%

“…Other risk factors include male sex, previous abdominal surgery, encephalopathy, ascites, a low platelet count and a history of bleeding varices [35,184,[194][195][196][197][198]. The role of inherited or acquired thrombophilia, as well as of previous sclerotherapy for variceal bleeding, is uncertain [35,184,[194][195][196][197][198].…”

Section: Portal Vein Thrombosis In Cirrhosismentioning

confidence: 99%

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Hemostatic balance in patients with liver cirrhosis: Report of a consensus conference

Andriulli

Tripodi

Angeli

et al. 2016

Digestive and Liver Disease

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a b s t r a c tPatients with cirrhosis present with hemostatic alterations secondary to reduced availability of procoagulant and anti-coagulant factors. The net effect of these changes is a rebalanced hemostatic system. The Italian Association of the Study of the Liver (AISF) and the Italian Society of Internal Medicine (SIMI) promoted a consensus conference on the hemostatic balance in patients with cirrhosis. The consensus process started with the review of the literature by a scientific board of experts and ended with a formal consensus meeting in Rome in December 2014. The statements were graded according to quality of evidence and strength of recommendations, and approved by an independent jury. The statements presented here highlight strengths and weaknesses of current laboratory tests to assess bleeding and thrombotic risk in cirrhotic patients, the pathophysiology of hemostatic perturbations in this condition, and outline the optimal management of bleeding and thrombosis in patients with liver cirrhosis.

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“…The most common abnormalities found in these patients were polymorphisms of methylene-tetrahydrofolate reductase and prothrombin gene. [29][30][31] 6. Correct answers: B, D and E Sinusoidal obstruction syndrome (SOS) is an obliterative venulitis of the terminal hepatic venules.…”

Section: Correct Answers: B and Dmentioning

confidence: 99%

Hepatobiliary Quiz Answers—19 (2016)

Rathi

Dhiman

2016

Journal of Clinical and Experimental Hepatology

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Inherited coagulation disorders in cirrhotic patients with portal vein thrombosis

Abstract: 677 =T were 13%, 34.8%, and 43.5% in cirrhotic patients with PVT and 7.5%, 2.5%, and 5% in cirrhotic patients without PVT, respectively. Five patients in the former group had associated defects. A thrombophilic genotype was detected in 69.5% of the patients with PVT.

Cited by 252 publications

References 38 publications

PAI-1 4G-4G, MTHFR 677TT, V Leiden 506Q, and Prothrombin 20210A in Splanchnic Vein Thrombosis: Analysis of Individual Patient Data From Three Prospective Studies

PAI-1 4G-4G, MTHFR 677TT, V Leiden 506Q, and Prothrombin 20210A in Splanchnic Vein Thrombosis: Analysis of Individual Patient Data From Three Prospective Studies

Hemostatic balance in patients with liver cirrhosis: Report of a consensus conference

Hepatobiliary Quiz Answers—19 (2016)

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