HYPERCOAGULABILITY IN PATIENTS WITH PRIMARY BILIARY-CIRRHOSIS AND PRIMARY SCLEROSING CHOLANGITIS EVALUATED BY THROMBELASTOGRAPHY

Background/Aims: Patients with primary biliary cirrhosis and primary s clerosing cholangitis survive variceal bleeding better than patients w ith alcoholic cirrhosis and have less bleeding at liver transplantatio n. Recently, patients with primary biliary cirrhosis have been found t o have a higher incidence of thrombosis in the portal venous tree, We hypothesized that primary biliary cirrhosis and primary sclerosing cho langitis patients may be hypercoagulable. Methods: We used thrombelast ography, which is a simple technique for evaluating whole blood clotti ng and fibrinolysis, to establish if hypercoagulability was present, d efined 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 fibri nogen levels), We evaluated 47 primary biliary cirrhosis and 21 primar y sclerosing cholangitis patients, 40 with non-cholestatic cirrhosis a nd 40 healthy subjects as control groups with thrombelastography, full blood count, prothrombin time, partial thromboplastin time and, fibri nogen concentrations, In those with hypercoagulability we evaluated pr otein 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%) primar y biliary cirrhosis and in nine of 21 (43%) primary sclerosing cholang itis patients independent of cirrhosis, and bilirubin concentration, b ut 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), Ther e was no correlation between the fibrinogen concentration (which was n ormal in all patients) or platelet count and the thrombelastography pa rameters, Only six of the 22 hypercoagulable patients had lower than n ormal values of protein S, C or antithrombin III. Activated protein C phenotype was normal in all. Conclusions: This difference between bili ary and parenchymal liver disease may have clinical implications, whic h need to be defined.