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Editorials |
1 Divisione di Ematologia, Ospedali Riuniti, L.go Barozzi 1, 24128 Bergamo, Italy
aAuthor for correspondence. Fax 39-035-266-667; e-mail ematologia@cyberg.it.
The antiphospholipid syndrome is a complex disorder (1) that is related to a set of unusual and fascinating antibodies. Recent research is defining these antibodies and their relationship to features of the syndrome.
The antiphospholipid syndrome is defined by the association of arterial and/or venous thrombosis, recurrent miscarriages, and anti-phospholipid (aPL) antibodies (1). The syndrome may be either idiopathic (primary) or may occur in the setting of an associated disease (secondary), most frequently systemic lupus erythematosus (SLE) or other autoimmune diseases (2)(3). Lupus anticoagulants and anti-cardiolipin antibodies at medium to high titers are laboratory indicators of the condition (4).
Lupus anticoagulants behave as acquired inhibitors of coagulation and prolong phospholipid-dependent in vitro coagulation tests (5), whereas anti-cardiolipin antibodies bind cardiolipin and other anionic phospholipids and are detected in solid-phase immunoassays (6). Despite their name, aPL antibodies recognize plasma proteins bound to suitable anionic (not necessarily phospholipid) surfaces rather than anionic phospholipids. Among them, ß2-glycoprotein I (ß2-GPI) (7)(8) and prothrombin (9) are the most common and investigated antigenic targets. ß2-GPI is required by the majority of autoimmune anti-cardiolipin antibodies to react with cardiolipin in immunoassays (7)(8). Specific subgroups of anti-ß2-GPI (10) and anti-prothrombin (11) antibodies are responsible for the lupus anticoagulant activity in phospholipid-dependent coagulation tests.
In addition to ß2-GPI and prothrombin,
(activated) protein C (12), protein S (12),
annexin V (13), high- and low-molecular weight kininogens
(14), factor XII (15), thrombomodulin
(16), and tissue-type plasminogen activator (17)
have been reported to be antigenic targets
References
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