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Abstract Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia which is characterized by recurrent thrombotic events and obstetric complications in the presence of Antiphospholipid antibodies (aPL). Pathogenic mechanisms include effects on the coagulation cascade, cellular activation and complement activation. Lately, much has been advanced in the knowledge of cellular receptors that participate in signaling transduction. The histopathological features of the antiphospholipid syndrome reflect a combination of several major pathophysiological processes: thrombotic microangiopathy, ischemia secondary to arterial thromboses or emboli, and peripheral embolization from venous, arterial, or intracardiac sources. Deep vein thrombosis is the most common venous event and stroke is the most common arterial event to occur in patients with APS. Other clinical features are recurrent miscarriage, fetal death, livedo reticularis, thrombocytopenia, cardiac valve disease and hepatic veno-occlusive disease. The diagnosis of APS is based on the discovery of one clinical manifestation and one laboratory criteria for circulating antiphospholipid antibodies at least detected at least twice in 12 weeks. The treatment of APS is based on anticoagulant properties, and benefits are weighed carefully against their significant risks. Life-long treatment with warfarin is standard for recurrent thrombotic events while for obstetric patients with APS, the standard therapy is subcutaneous Low-molecular-weight heparin (LMWH) and low-dose aspirin. Further studies are needed to clarify how aPL Abs affect cell surface molecules and how signal transduction events occur. Understanding intracellular events in aPL-mediated endothelial cell (EC), platelet and monocyte activation may help in designing new targeted therapies for thrombosis in APS. Conclusion: APS is a multifaceted and complex condition which is clinically demanding with respect to both diagnosis and management. There is a clear need for further studies into the pathogenetic mechanisms involved, for the development of more specific laboratory techniques to identify those patients at particular risk of thrombosis and miscarriage and for the enrolment of patients in prospective controlled trials of treatment. |