Laboratory evaluation of the antiphospholipid syndrome.
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 (beta2GPI). In the intervening years, it has been established that beta2GPI 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-beta2GPI for the classification of APS.
Abo
Stephen M
SM
Division of Hematology and Oncology, Department of Internal Medicine, Seton Hall University, School of Graduate Medical Education, 400 South Orange Ave., South Orange, 07079, and St. Joseph's Regional Medical Center, Paterson, NJ, USA.
DeBari
Vincent A
VA
D017153:Antibodies, Anticardiolipin / Q000378:metabolism; D017152:Antibodies, Antiphospholipid / Q000378:metabolism; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000378:metabolism*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000378:metabolism; D053482:beta 2-Glycoprotein I / Q000378:metabolism
D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid; D016682:Lupus Coagulation Inhibitor; D053482:beta 2-Glycoprotein I