Antiphospholipid thrombosis syndromes.
Antiphospholipid antibodies are associated strongly with thrombosis and are the most common of the acquired blood protein defects causing thrombosis. Although the precise mechanisms whereby antiphospholipid antibodies alter hemostasis to induce a hypercoagulable state remain unclear, numerous theories, as previously discussed, have been advanced. The most common thrombotic events associated with ACLAs are deep vein thrombosis and pulmonary embolus (type I syndrome), coronary or peripheral artery thrombosis (type II syndrome), or cerebrovascular/retinal vessel thrombosis (type III syndrome), and occasionally patients present with mixtures (type IV syndrome). Patients with type V disease are those with antiphospholipid antibodies and RMS. It is as yet unclear how many seemingly normal individuals who may never develop manifestations of antiphospholipid syndrome (type VI) harbor asymptomatic antiphospholipid antibodies. The relative frequency of ACLAs in association with arterial and venous thrombosis strongly suggests that they should be looked for in any individual with unexplained thrombosis; all three idiotypes (IgG, IgA, and IgM) should be assessed. Also, the type of syndrome (I-VI) should be defined, if possible, because this identification may dictate both the type and the duration of immediate and long-term anticoagulant therapy. Unlike those patients with ACLAs, patients with primary LA-thrombosis syndrome usually have venous thrombosis. Because the aPTT is unreliable in patients with LA (prolonged in only approximately 40%-50% of patients) and usually is not prolonged in patients with ACLAs, definitive tests, including ELISA for ACLA, the dilute Russell's viper venom time for LA, hexagonal phospholipid-neutralization procedure, and B-2-GP-I (IgG, IgA, and IgM) should be ordered immediately when suspecting antiphospholipid syndrome or in individuals with otherwise unexplained thrombotic or thromboembolic events. If these test results are negative, subgroups also should be assessedin the appropriate clinical setting. Most patients with antiphospholipid thrombosis syndrome will fail to respond to warfarin therapy, and except for retinal vascular thrombosis, may fail some types of antiplatelet therapy, so it is of major importance to make this diagnosis so patients can be treated with the most effective therapy for secondary prevention-LMWH or unfractionated heparin in most instances and clopidogrel in some instances.
Bick
Rodger L
RL
Department of Medicine and Pathology, University of Texas Southwestern Medical Center, 10455 North Central Expressway, Suite 109-PMB320, Dallas, TX 75231, USA. rbick@thrombosis.com
D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D013927:Thrombosis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy