Arterial and venous thromboses occurring in young women and, less commonl3~ men without an obvious cause, e.g. presence of risk factors for premature atherosclerosis or blood dyscrasias, have long been known to vascular surgeons. The recognition of autoimmune disorders such as systemic lupus erythematosus (SLE), seemed to explain many of these occurrences on the basis of vasculitis causing thrombosis. However not all such patients had SLE and, even in those that did, there seemed to be a subset in whom the disease predominantly caused thrombosis, rather than the more usual clinical features of arthropathy and nephropathy. The first clue to the existence of the anti phospholipid syndrome as the cause of these thromboses was the discovery of the lupus anticoagulant (LAC) in 1952. 2 This was an in vitro coagulation inhibitor found in the plasma of patients with SLE, hence the name lupus anticoagulant. Paradoxically, however, later these patients with lupus anticoagulant were shown to be at a high risk of thrombosis rather than haemorrhage. 2 Patients with LAC also often had a positive Wassermann reaction the so called biological false positive Wassermann 3 suggesting, as the Wassermann reaction depends on binding of syphilis antibody to phospholipid, an abnormality of phospholipid in these patients. In 1983 direct measurement of an anti phospholipid antibody (APL) was described by Harris et al. 4 It seemed that the lupus anticoagulant and APL antibody were similar, though not identical antibodies to phospholipid and the anti phospholipid syndrome (APLS) was recognised, s The presence of APL antibody in young patients with arterial and venous thrombosis was confirmed in many studies over the next decade. 6 It soon became apparent that not all patients with APLS had SLE and that there appeared to be a primary form of the syndrome. 7 Also the antibody was found, often transiently, in many other disease states such as streptococcal infection, malignancy, other autoimmune disorders and use of certain drugs. The association of presence of antibody and thrombosis is less clear in these situations. 6 The main features of the APLS are venous and arterial thrombosis, particularly cerebral, recurrent foetal loss, thrombocytopenia and skin lesions such as livedo reticularis. Many other clinical features such as cardiac disease and other neurological disorders have also been described. Diagnostic criteria are not completely established but the occurrence of two or more thrombotic events in association with levels of APL antibody several times the normal, on two or more occasions over several weeks, may be taken as diagnostic s The two laboratory tests used in the diagnosis of the condition are the demonstration of LAC in the plasma and of raised levels of APL. Presence of LAC depends on first showing an abnormality, i.e. prolongation, of an in vitro phospholipid dependent test of the intrinsic coagulation pathwa~ second that the abnormality is due to the presence of an inhibitor and third that the inhibitor is directed at phospholipid. The activated partial thromboplastin time (APTT), kaolin clotting time (KCT) or dilute Russell viper venom test (DRVVT) can be used first. 68 Presence of an inhibitor is shown by mixing studies using normal platelet free plasma and confirmation that the inhibitor is directed against phospholipid can be shown by use of an exogenous phospholipid source, e.g. freeze thawed platelets, to reverse the abnormality. If the patient is on oral anticoagulants, as is often the case when the syndrome is first suspected, testing for lupus anticoagulant is difficult, needing complex laboratory manoeuvres. s Direct demonstration of raised levels of the
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