Our results show that high-risk subjects with triple-positive aPL profiles are identified early, at the time of the initial screening tests.

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Venous or arterial thrombosis or pregnancy morbidity in the presence of circulating antiphospholipid antibodies (aPL) define the antiphospholipid syndrome (APS). In terms of accepted APS criteria, aPL are detected by one coagulation test (lupus anticoagulant; LAC) and two immunoassays (anticardiolipin antibodies and anti-β2-glycoptrotein I antibodies). In patients with APS, a single positive test carries a much lower risk of thrombosis recurrence or new pregnancy loss than does multiple (or triple) positivity. The same holds true for aPL carriers, namely subjects with laboratory tests but without clinical criteria for APS. Thus, very different risk categories exist among patients with APS as well as in carriers of aPL. Triple positivity apparently identifies the pathogenic autoantibody (antidomain I-II of β2-glycoptrotein I); it is in this category of patients that trials on new therapeutic strategies should focus.

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Summary This is a practical report on laboratory tests for the diagnosis of antiphospholipid syndrome (APS). After a general definition of APS, this study deals with appropriateness and timing in requesting the determination of antiphospholipid (aPL) antibodies. Lupus anticoagulant (LAC), anticardiolipin (aCL), and anti β2‐glycoprotein I (aβGPI) are the mandatory tests to be performed, while other tests are not yet validated for clinical use. Interpretation of results is an important discussed issue that implies a close liaison between clinical pathologists and clinicians. Finally, a personal definition of APS according to aPL antibody profile closes the manuscript.