Vol. 1, Issue 1 (2015)
Hematological perspective of antiphospholipid antibody syndrome - An updated overview
Author(s): Mirza Asif Baig
Abstract: Background: The antiphospholipid antibody syndrome (APS) is a systemic, acquired, immune-mediated disorder characterized by episodes of venous, arterial, or microcirculation thrombosis and/or pregnancy abnormalities, associated with the persistent presence of autoantibodies, confirmed at least in two occasions 12 weeks apart, directed to molecular complexes consisting of phospholipids and proteins. It comprises 1% of general population.rnDiscussion: APS is a prothrombotic condition characterized by the presence of a lupus anticoagulant (LA), anticardiolipin antibodies (aCL) and anti-β2-glycoprotein-1 antibodies in patients with recurrent pregnancy morbidity and/or thromboembolic complications. Thrombocytopenia is afrequent finding in patients with APS, and balancing the need for anticoagulationو when faced with significant thrombocytopenia can be a considerable challenge for clinicians managing such patients.rnAPS can cause preeclampsia (18%), pregnancy-induced hyper¬tension, foetal death (7%), retardation (31%), premature labor (43%), stillbirth, and ultimately sterility. It is generally recommended that a panel of tests be done for diagnosis of APS. Patients who test positive for all three of the major assays—positive LAC, elevated anticardiolipin antibodies and elevated anti- beta 2GPI antibodies (referred to as “triple positivity”), are at markedly increased risk for thrombosis and for pregnancy complications.rnConclusion: APS is a prothrombotic disorder with various manifestations, most commonly venous and arterial thromboembolism and recurrent pregnancy loss. Diagnosis of APS can be challenging due to evolving criteria, potential limitations in the laboratory assays used to measure antiphospholipid antibodies and overlapping characteristics with other prothrombotic thrombocytopenic disorders. APS should always be considered as a potential diagnosis especially for young patients presenting with a history of thrombotic events, in particular when they occur without any obvious external trigger conditions or/and for women with recurrent pregnancy loss or later fetal deaths. Many other disorders are able to mimic APS, so a broad range of alternative diagnoses should be investigated and ruled out during clinical workup.