Search

In Western countries, hepatic vein thrombosis (Budd-Chiari syndrome) is, as a rule, an uncommon though well-known complication of acquired or hereditary thrombophilia. It may be complicated by overt defibrination when secondary to deep venous thrombosis ascending to the vena cava. To be effective treatment should consist of early initial intensive thrombolysis for up to one week, followed without interruption by an APTT-controlled heparin infusion. Oral anticoagulant therapy is started together with the heparin, which should not be discontinued until the prothrombin times, expressed as international normalized ratios (INR), lie within a therapeutic range of 3-5 INR for 2 days, the target being 4 INR. After this intensity of anticoagulation has been maintained for three weeks, the range may be lowered to 2.5-4 INR, the target being 3 INR. Oral anticoagulation should be continued for as long as thrombophilia persists.

Appropriate long-term oral anticoagulation prevents cardiogenic thromboembolism to a large extent in patients with artificial heart valves, rheumatic heart disease, myocardiopathy, atrial fibrillation of non-rheumatic origin, sick sinus syndrome, cardiac aneurysm, and in the exceptional cases of mitral valve prolapse with thromboembolic complications. In arterial thrombosis, oral anticoagulation remains a controverted, probably because a much higher intensity would be needed to achieve the same degree of effectiveness. With target prothrombin times between 3.5 and 4 International Normalized Ratios (INRs) and a compliance of the INRs with the range of 2.5-5 INRs for greater than or equal to 80%, cardiogenic thromboemboli can be prevented in approximately 95% whereas only about two thirds of the cases of recurrent coronary thrombosis can be avoided. The intensity and stability of treatment needed in cardiovascular thrombosis involve a considerable risk of bleeding, but--as shown by the results of the Dutch Sixty Plus Reinfarction Study--intracranial haemorrhages are more than compensated for by the prevention of cerebrovascular thromboembolic events. Appropriate administration of oral anticoagulation requires painstaking laboratory and therapeutic control, the former being based on continuous quality assessment and strict standardization of the prothrombin time. Therapeutic control consists of continuous patient education and adequate dosage regulation. Similar to the situation prevailing for hemophilia patients, an organization must be available to which long-term anticoagulated patients can apply for expert advice. In The Netherlands, an organization has been built up on a voluntary basis, called Federation of Thrombosis Centres, meeting this requirement and covering more than 90% of the country.

Protein C is a vitamin K-dependent protein which is produced in the liver. Activated protein C has an anticoagulant effect by inactivating the clotting factors V and VIII. We report on a young female patient who suffered from recurrent thrombosis of the deep calf and pelvic veins with pulmonary embolism. Superficial thrombophlebitis also occurred repeatedly. In the plasma of this patient we found reduced levels of protein C antigen (0.62 U/ml) and activity (0.42 U/ml). Investigation of other family members revealed a protein C deficiency in her father, sister, and son. During the anticoagulant treatment with Marcumar the patient developed a coumarin-induced skin necrosis, to which complication a protein C deficiency is evidently predisposed. This complication could only be prevented if heparin was given simultaneously during the adjustment period.