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Annals of Clinical & Laboratory Science 37:3-14 (2007)
© 2007 Association of Clinical Scientists


Review

Laboratory Evaluation of the Antiphospholipid Syndrome

Stephen M. Abo and Vincent A. DeBari
Division of Hematology and Oncology, Department of Internal Medicine, School of Graduate Medical Education, Seton Hall University, South Orange, New Jersey; St. Joseph’s Regional Medical Center, Paterson, New Jersey; and St. Michael’s Medical Center, Newark, New Jersey

Address correspondence to Vincent A. DeBari, Ph.D., Seton Hall University, School of Graduate Medical Education, 400 South Orange Ave., South Orange, NJ 07079; e-mail debarivi{at}shu.edu.

The antiphospholipid syndrome (APS) was first described in 1986. The original association of this hypercoagulable state with anticardiolipin antibodies (aCL) resulted from the synthesis of evidence stemming from laboratory findings in systemic lupus erythematosus (SLE), ie, the frequent occurrence of false-positive VDRL tests and the paradoxical observation of the so-called "lupus anticoagulant" (LA), an increase in phospholipid (PL)-dependent clotting times. By the early 1990s, it was clear that a co-factor was involved in the reaction of antibodies to PL (aPL) in SLE patients with secondary APS and that this was a hitherto-obscure protein, beta-2 glycoprotein I (ß2GPI). In the intervening years, it has been established that ß2GPI and other PL-binding proteins such as prothrombin (PT) are relevant antigens in APS and assays for these antigens have been developed, standardized, and applied to subjects with both primary and secondary APS. Measurement and confirmation of LA activity is based on a stepwise approach and should follow the recommendations of the International Society of Thrombosis and Haemostasis. Although antibodies to various PL-binding proteins have been suggested as diagnostic targets for APS, the current (2006) consensus guidelines recognize only LA, aCL, and anti-ß2GPI for the classification of APS.

Keywords: antiphospholipid syndrome, Hughes’ syndrome, lupus anticoagulant, anticardiolipin antibodies, phospholipid-binding proteins, beta-2 glycoprotein I, systemic lupus erythematosus







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