GUIDELINES FOR THE MANAGEMENT OF THROMBOPHILIA

Although there are numerous risk factors for venous thromboembolic dis ease, the term 'thrombophilia' refers only to those familial or acquir ed disorders of the haemostatic system that result in an increased ris k of thrombosis. The inherited thrombophilias include antithrombin III deficiency, resistance to activated protein C (factor V Leiden), prot ein C and protein S deficiencies as well as some rare forms of dysfibr inogenaemia. It is possible that other inherited conditions might also predispose to thrombosis. In contrast, when using the above definitio n, the antiphospholipid syndrome is the only genuine acquired thrombop hilic state. Patients who have thromboembolic disease ata young age wi th no provoking event or who have a positive family history or whose t hrombosis involves an unusual site should be investigated for thrombop hilia. The management of a patient identified as having a laboratory a bnormality associated with thrombophilia will depend on a variety of f actors such as the patient's individual and family thrombotic history, the site of the thrombosis and the presence of other prothrombotic ri sk factors. The use of prophylactic anticoagulation during pregnancy a nd the puerperium requires particularly careful consideration in throm bophilic women. As more becomes known about the thrombophilias it will become possible to formulate more exact guidelines as to the manageme nt of these conditions.