Antiphospholipid Syndrome

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The page below is a sample from the LabCE course Laboratory Evaluation of the Lupus Anticoagulant found in Antiphospholipid Syndrome (APS). Access the complete course and earn ASCLS P.A.C.E.-approved continuing education credits by subscribing online.

Learn more about Laboratory Evaluation of the Lupus Anticoagulant found in Antiphospholipid Syndrome (APS) (online CE course)
Antiphospholipid Syndrome

Antiphospholipid syndrome (APS) is condition in which a heterogeneous group of antibodies, IgG, IgM or both, are produced that are directed against phospholipid-protein complexes. Antiphospholipid antibodies found in Antiphospholipid Syndrome include lupus anticoagulants (LA) , anti-cardiolipin antibodies (ACA). and anticardiolipin beta 2 glycoprotein (anti-B2 GP1) complexes. Identified in the mid-1980s by Hughes as a condition associated with Systemic Lupus Erythematosus it has more recently been recognized to occur in three forms. Primary Antiphospholipid Syndrome (Primary APS) occurs in patients with no other systemic autoimmune disorder, associated APS occurs in patients with other systemic autoimmune disorders such as SLE and Rheumatoid Arthritis (RA) and catastrophic APS is when there is multiple organ thrombosis and failure with a mortality rate of more than 50%.

Within the larger classification of APS, two additional syndromes have been identified: the Anticardiolipin Antibody (aCL) syndrome and the Lupus Anticoagulant Syndrome (LAC). Both syndromes can be associated with thrombosis, thrombocytopenia or fetal loss.
The prevalence of APS is approximately 5% in the general population and increases with age. Antibodies linked to thromboembolic events are those that are directed toward anionic phospholipids. One proposed mechanism suggests that annexin A2 meditates endothelial cell activation by antiphospholipids and anti-beta 2 glycoprotein 1 antibodies. Normally annexin V prevents the formation of coagulation complexes on the phospholipid surface. Disruption of annexin V by the antiphospholipids could lead to endothelial cell activation with increased coagulation complex formation and thrombosis.