Spectrum of hemostatic derangements, in Budd-Chiari syndrome.

BACKGROUND: Hemostatic abnormalities have been reported in various hepatocellular diseases. We evaluated the hemostatic functions in patients with Budd-Chiari syndrome. METHODS: Biochemical liver function tests, and measurement of prothrombin time, activated partial thromboplastin time, and plasma levels of anti-thrombin III (antigen) and activity of protein C were done in 36 patients with Budd-Chiari syndrome. RESULTS: Liver biochemistry was abnormal in 34 patients. Plasma prothrombin time and activated partial thromboplastin time were prolonged in 17 (47%) and 23 (64%) patients, respectively. Antithrombin III antigen levels and protein C activity were reduced in 15 (50%) and 25 (83%) patients, respectively, among the 30 patients studied. Albumin levels showed significant correlation with coagulation test results, levels of anti-thrombin-III, and protein C activity. CONCLUSION: Hepatic synthesis of coagulation factors and anticoagulants is reduced in Budd-Chiari syndrome; this may play a role in recurrence of thrombosis.

Hemostatic alterations in non-cirrhotic portal fibrosis, extrahepatic portal venous obstruction and Budd-Chiari syndrome.

We studied the coagulation function in ten patients each with non-cirrhotic portal fibrosis (NCPF), extrahepatic portal venous obstruction (EHPO) and Budd-Chiari syndrome (BCS), conditions where venous thrombosis in the hepatic vasculature is a common denominator. Prothrombin time, partial thromboplastin time with kaolin (PTTK) and thrombin time were normal in patients with NCPF and EHPO. However, in BCS the PTTK was prolonged, with a mean test/control ratio of 1.68 +/- 0.11. Fibrin degradation products were absent in all patients. Platelet aggregation tests showed hypoaggregability in all patients with NCPF. They were normal in patients with EHPO. However, two of ten BCS patients showed hyperaggregability, coinciding with a recent onset of illness in one patient. In conclusion, coagulation abnormalities appear unlikely to be the cause of thrombosis in patients with NCPF and EHPO. Further studies are required to substantiate the findings of hyperaggregability of platelets in BCS.