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Hypercoagulability in patients with primary biliary cirrhosis and primary sclerosing cholangitis evaluated by
Z Ben-Ari1, M Panagou, D Patch
1Liver Transplantation & Hepatobiliary Medicine, Royal Free Hospital, London, UK.
Insights
Patients with primary biliary cirrhosis and primary sclerosing cholangitis show increased hypercoagulability, indicating a higher risk of thrombosis. This contrasts with non-cholestatic cirrhosis patients, suggesting distinct disease mechanisms and potential clinical implications for cholestatic liver diseases.
Area of Science:
- Hepatology
- Hematology
- Clinical Biochemistry
Background:
- Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) patients exhibit better survival from variceal bleeding and less bleeding during liver transplantation compared to alcoholic cirrhosis patients.
- PBC patients have a higher incidence of portal venous tree thrombosis.
- This suggests a potential hypercoagulable state in PBC and PSC.
Purpose of the Study:
- To investigate the presence of hypercoagulability in patients with PBC and PSC.
- To compare the hypercoagulability status between cholestatic liver diseases and non-cholestatic cirrhosis.
Main Methods:
- Thrombelastography (TEG) was used to assess whole blood clotting and fibrinolysis.
- Hypercoagulability was defined by specific TEG parameters (r, maximum amplitude, alpha angle) exceeding normal control ranges.
- Evaluated 47 PBC, 21 PSC, 40 non-cholestatic cirrhosis patients, and 40 healthy controls, alongside coagulation factor levels in hypercoagulable individuals.
Main Results:
- Hypercoagulability, defined by three TEG abnormalities, was present in 28% of PBC and 43% of PSC patients.
- Significantly fewer non-cholestatic cirrhosis patients (5%) and no healthy controls exhibited hypercoagulability (p<0.03 and p<0.0002).
- No correlation was found between TEG parameters and fibrinogen concentration or platelet count; coagulation factor levels were mostly normal in hypercoagulable patients.
Conclusions:
- Patients with PBC and PSC demonstrate a higher prevalence of hypercoagulability compared to those with non-cholestatic cirrhosis.
- This hypercoagulable state in biliary liver diseases may contribute to thrombosis.
- The observed differences highlight distinct pathophysiological mechanisms between biliary and parenchymal liver diseases, warranting further clinical investigation.
Background/Aims:
Patients with primary biliary cirrhosis and primary sclerosing cholangitis survive variceal bleeding better than patients with alcoholic cirrhosis and have less bleeding at liver transplantation. Recently, patients with primary biliary cirrhosis have been found to have a higher incidence of thrombosis in the portal venous tree. We hypothesized that primary biliary cirrhosis and primary sclerosing cholangitis patients may be hypercoagulable.
Methods:
We used thrombelastography, which is a simple technique for evaluating whole blood clotting and fibrinolysis, to establish if hypercoagulability was present, defined by thrombelastography values greater than 2SD over controls: r<19 mm (this reflects plasma clotting factors), maximum amplitude (ma) >60 mm, and alpha angle >43 degrees (these reflect platelets and fibrinogen levels). We evaluated 47 primary biliary cirrhosis and 21 primary sclerosing cholangitis patients, 40 with non-cholestatic cirrhosis and 40 healthy subjects as control groups with thrombelastography, full blood count, prothrombin time, partial thromboplastin time and, fibrinogen concentrations. In those with hypercoagulability we evaluated protein S, C, anti-thrombin III levels and activated protein C phenotype.
Results:
All three thrombelastography abnormalities present together defined hypercoagulability: these were found in 13 of 47 (28%) primary biliary cirrhosis and in nine of 21 (43%) primary sclerosing cholangitis patients independent of cirrhosis, and bilirubin concentration, but in only 2 of 40 (5%) patients with noncholestatic cirrhosis and in none of the healthy controls (p<0.03 and p<0.0002, respectively). There was no correlation between the fibrinogen concentration (which was normal in all patients) or platelet count and the thrombelastography parameters. Only six of the 22 hypercoagulable patients had lower than normal values of protein S, C or antithrombin III. Activated protein C phenotype was normal in all.
Conclusions:
This diffference between biliary and parenchymal liver disease may have clinical implications, which need to be defined.