Antiphospholipid syndrome

Service of Internal Medicine, Hospital de Cruces-University of the Basque Country, Bizkaia, Spain.
The Lancet (Impact Factor: 39.21). 10/2010; 376(9751):1498-509. DOI: 10.1016/S0140-6736(10)60709-X
Source: PubMed

ABSTRACT The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs.

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    ABSTRACT: Corticosteroid or intravenous immunoglobulin is used in the management of idiopathic thrombocytopenic purpura during pregnancy. A patient with idiopathic thrombocytopenic purpura had a previous history of interrupted pregnancy due to severe thrombocytopenia, and was unresponsive to high doses of corticosteroids and intravenous immunoglobulin. Immediately following pregnancy, our patient had a marked elevation in plasma levels of fibrinogen degradation products, D-dimer, and platelet factor 4, with a decrease in platelets, suggesting platelet activation and thrombogenesis. Combined treatment with an anticoagulant agent could prevent exacerbation of thrombocytopenia throughout pregnancy. Although the underlying causes leading to the series in her pregnancy course were uncertain, there were notable serological abnormalities, such as weakly positive antinuclear antibody and anti-U1-RNP antibody. When thrombocytopenia rapidly develops in patients with idiopathic thrombocytopenic purpura immediately following pregnancy, the possibility of a thrombogenic state and differential diagnosis, including antiphospholipid syndrome and collagen vascular disease, should be considered. Treatment with an anticoagulant agent might then be appropriate.
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    ABSTRACT: The antiphospholipid syndrome is characterized by a combination of laboratory findings (i.e., the presence of at least one antiphospholipid antibody) and clinical manifestations (arterial and/or venous thrombosis, obstetrical complications). Long-term oral anticoagulant is recommended to prevent recurrence of both arterial and venous thrombosis, whereas (low molecular weight) heparin plus aspirin is the treatment of choice to prevent further obstetrical complications. In the rare case of catastrophic antiphospholipid syndrome, heparin plus high-dose corticosteroids plus plasma exchange is associated with the highest recovery rate. Some new, non-antithrombotic-based treatments of antiphospholipid syndrome with rituximab, autologous stem cell transplantation, or hydroxychloroquine are also reviewed.
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    ABSTRACT: Elective therapeutic approaches are required since recurrent thrombosis remains a major challenge in the management of antiphospholipid syndrome (APS) despite an efficient anticoagulation. Several data suggest that hydroxychloroquine (HCQ) could play a role in the prevention of thrombosis. The goal of this review is to point out the different aspects that could suggest the usefulness and the efficacy of HCQ for the prevention of thrombosis relapse in APS. By Medline research we collected important data dealing with potential anti-thrombotic effects of HCQ. The mechanisms of action of HCQ, and clinical and experimental data in systemic lupus erythematosus (SLE) and APS are discussed. As HCQ reduces the risk of thrombosis in both SLE patients and animal models of APS (1-7), and possibly decreases the titre of aPL (8), its beneficial role as a potential antithrombotic could be suggested. Copyright © 2014 Elsevier B.V. All rights reserved.
    Autoimmunity Reviews 12/2014; 14(4). DOI:10.1016/j.autrev.2014.12.006 · 7.10 Impact Factor

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