Thrombophilic conditions in non-cirrhotic portal vein thrombosis (original) (raw)
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Portal vein thrombosis (PVT): A study of 20 non-irrhotic cases
Swiss medical weekly: official journal of the Swiss Society of Infectious Diseases, the Swiss Society of Internal Medicine, the Swiss Society of Pneumology
Portal and mesenteric venous thrombosis (PVT) is an uncommon disease with serious consequences if not discovered early in order to prevent complications such as variceal bleeding and intestinal ischaemia. The objective of this study was to describe the clinical presentation and outcome of patients with PVT with a view to early diagnosis and treatment of this disease. The study was restricted to patients with PVT not caused by underlying liver cirrhosis. To analyse important clinical characteristics of this entity we performed a retrospective study of 20 non-cirrhotic patients seen in our hospital from February 1998 to March 2003. The main clinical symptom was abdominal pain (13 patients, 86%), sometimes in combination with diarrhoea and vomiting (5 patients, 33%), nausea and anorexia (3 patients). Laboratory signs were non-specific and diagnosis was usually by computed tomography (19 patients, 95%). Causative factors included prothrombotic states (9 patients, 45%) and/or local facto...
Portal vein thrombosis: A concise review (Review)
Experimental and Therapeutic Medicine, 2021
Portal vein thrombosis (PVT) is a frequent complication in cirrhotic patients, but it may also exist as a basic vascular condition even without any liver damage. Local and systemic factors play a significant role in the pathogenesis of PVT; yet, in practice, more than one factor may be identified. PVT can be considered a result of liver fibrosis and hepatic insufficiency. The JAK2 mutation has been accepted as a factor producing PVT. In general, the anticoagulants are recommended but this therapy should be used carefully in treating patients that associate coagulopathy or thrombocytopenia and esophageal varices. Acute PVT without bowel infarction has a good prognosis. In liver cirrhosis, the mortality due to hemorrhage is higher than in chronic PVT. Therefore, for the patients with PVT, the survival rate is decreased by 55% in two years, due to hepatic insufficiency. Regarding the treatment, LMWH (low molecular weight heparine) is the most utilized in patients with cirrhosis, non-malignancies, infections, or those who are awaiting a liver transplant. DOACs (direct-acting oral anticoagulants) may be used in the rest of the medical conditions, being safe and equal to LMWH.
Portal vein thrombosis: Insight into physiopathology, diagnosis, and treatment
World Journal of Gastroenterology, 2010
Portal vein thrombosis (PVT) is a relatively common complication in patients with liver cirrhosis, but might also occur in absence of an overt liver disease. Several causes, either local or systemic, might play an important role in PVT pathogenesis. Frequently, more than one risk factor could be identified; however, occasionally no single factor is discernable. Clinical examination, laboratory investigations, and imaging are helpful to provide a quick diagnosis, as prompt treatment might greatly affect a patient's outcome. In this review, we analyze the physiopathological mechanisms of PVT development, together with the hemodynamic and functional alterations related to this condition. Moreover, we describe the principal factors most frequently involved in PVT development and the recent knowledge concerning diagnostic and therapeutic procedures. Finally, we analyze the implications of PVT in the setting of liver transplantation and its possible influence on patients' future prognoses.
Hypercoagulability in Patients With Chronic Noncirrhotic Portal Vein Thrombosis
Clinical Gastroenterology and Hepatology, 2012
Although they have normal liver histology and function, patients with chronic noncirrhotic nontumoral portal vein thrombosis (NC-PVT) frequently have abnormal results from coagulation tests. We investigated the significance of these results. METHODS: We analyzed blood samples collected from 50 stable patients with NC-PVT secondary to a thrombophilic disorder (32%) or local factor (32%), or that was idiopathic (36%). We measured endogenous thrombin potential (ETP) with and without thrombomodulin, prothrombin time, activated partial thromboplastin time, coagulation factors (I, II, V, VII, VIII, IX, X, XI, and XII), antithrombin, proteins C and S, von Willebrand factor (vWF) antigen, vWF ristocetin cofactor, a disintegrin and metalloprotease with thrombospondin type 1 motifs 13 antigen, D-dimer, plasminantiplasmin complex, prothrombin fragment F1ϩ2, activated factor VII, and clot lysis time. Samples from 50 age-and sexmatched healthy individuals were evaluated as controls. RE-SULTS: Compared with controls, patients with NC-PVT had significant increases in prothrombin time and activated partial thromboplastin time; they had significant reductions in levels of procoagulant factors II, V, VII, IX, X, XI, and XII, and the anticoagulants antithrombin, protein C, and protein S. The patients had increased levels of factor VIII and vWF antigen. Irrespective of etiology, patients with NC-PVT had a significant increase in ETP with thrombomodulin and higher levels of factor VIIa, prothrombin fragment F1ϩ2, D-dimer, and plasmin-antiplasmin complex than controls, indicating in vivo activation of coagulation and fibrinolysis. CONCLUSIONS: Patients with NC-PVT have hypercoagulability that is independent of the underlying etiology, based on in vitro analyses of thrombin-generation capacity and increased levels of biomarkers in blood samples. Further studies are required to determine if activation of hemostasis increases the risk for thrombotic events.
1. PORTAL VEIN THROMBOSIS ETIOLOGY, DIAGNOSIS AND MANAGEMENT
Portal Vein Thrombosis (PVT) is a common clinical problem often found in Gastroenterology Clinics. It may occur with or without a pre-existing chronic liver disease. Clinical course may be acute or chronic. Clinical features vary in acute and chronic Portal Vein Thrombosis. Acute PVT usually presents with pain abdomen while as chronic PVT presents with features of Portal Hypertension. Management also differs-acute PVT is managed with anticoagulants while as chronic PVT is managed as portal hypertension.
Journal of Thrombosis and Haemostasis, 2007
Medicine Group. Homocysteine and markers of coagulation and endothelial cell activation. J Thromb Haemost 2004; 2: 445-51. 9 Homocysteine Lowering TrialistsÕ Collaboration. Dose-dependent effects of folic acid on blood concentrations of homocysteine: a metaanalysis of the randomized trials. Am J Clin Nutr 2005; 82: 806-12. 10 Vermeulen EG, Rauwerda JA, van den Berg M, de Jong SC, Schalkwijk C, Twisk JW, Stehouwer CD. Homocysteine-lowering treatment with folic acid plus vitamin B6 lowers urinary albumin excretion but not plasma markers of endothelial function or C-reactive protein: further analysis of secondary end-points of a randomized clinical trial. Eur J Clin Invest 2003; 33: 209-15. 11 Klerk M, Durga J, Schouten EG, Kluft C, Kok FJ, Verhoef P. No effect of folic acid supplementation in the course of 1 year on haemostasis markers and C-reactive protein in older adults. Thromb Haemost 2005; 94: 96-100. 12 Peeters ACTM, van der Molen EF, Blom HJ, den Heijer M. The effect of homocysteine reduction by B-vitamin supplementation on markers of endothelial dysfunction. Thromb Haemost 2004; 92: 1086-91.
Cause of portal or hepatic venous thrombosis in adults: The role of multiple concurrent factors
Hepatology, 2000
According to a recent hypothesis, venous thrombosis results from the concurrence of several factors. This hypothesis was assessed in patients with portal or hepatic venous thrombosis by simultaneously investigating most of the currently identified prothrombotic disorders, local precipitating factors, and other risk factors such as oral contraceptive use. Patients with a tumorous obstruction and patients with cirrhosis with portal vein thrombosis were excluded. The prothrombotic disorders that were investigated included classical and occult myeloproliferative disorders; antiphospholipid syndrome; protein C; protein S and antithrombin deficiency; factor V Leiden; factor II; and methylene-tetrahydrofolate-reductase gene mutations. We found 1 or several prothrombotic disorders and a local precipitating factor in 26 and 10 of the 36 patients with portal vein thrombosis, respectively; and in 28 and none of the 32 patients with hepatic vein thrombosis, respectively. We found a combination of prothrombotic disorders in 5 and 9 patients with portal and hepatic vein thrombosis, respectively, whereas such a combination is expected in less than 1% of asymptomatic subjects. Of the 10 patients with a local precipitating factor, 8 had a prothrombotic disorder. Of the 13 patients who use oral contraceptives, 10 had a prothrombotic disorder. We conclude that portal or hepatic venous thrombosis should be regarded as an index for 1 or several prothrombotic disorders, whether or not local precipitating factors or oral contraceptive use are found. Concurrence of prothrombotic disorders is more common than expected. Extensive investigation of prothrombotic disorders and anticoagulation should be considered in patients with portal or hepatic venous thrombosis. (HEPATOLOGY 2000;31: 587-591.) According to a recent concept, venous thrombosis would result from the convergence of an inherited predisposition, owing to a mutation in 1 or more genes, and an acquired Abbreviation: MTHFR, methylene-tetrahydrofolate reductase.
Portal Vein Thrombosis: A Case report and Literature Review
A case report of Portal Vein Thrombosis (PVT) as a complication of protein S deficiency. PVT has been increasingly diagnosed over the years, particularly through the use of ultrasound-Doppler equipment. The lifetime risk of getting PVT in the general population has recently reported to be 1%.1 While this condition has traditionally been associated with cirrhosis or liver malignancy, it may also occur without any liver disease. The case report is followed by a discussion of the aetiology and clinical presentations of PVT, as well as a review of the investigations and management proposed in the literature.
Portal Thrombosis in Cirrhosis: Role of Thrombophilic Disorders
Journal of Clinical Medicine, 2020
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 thromboph...