Cases reported "Antiphospholipid Syndrome"

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11/336. Spontaneous venous thrombosis in a young patient with combined factor v Leiden and lupus anticoagulant.

    We describe a case of a 28-year-old man who developed an extensive spontaneous deep venous thrombosis. Testing revealed heterozygotic factor v Leiden mutation, and the presence of both lupus anticoagulant (LA) and elevated IgM anticardiolipin antibody (ACA). Several family members were found to be heterozygous for factor v Leiden. A paternal aunt had the factor v Leiden mutation, an elevated plasma homocysteine and a borderline increased IgG ACA level. No other family member had a history of a venous thrombotic event. This case illustrates that evaluation of young patients who present with venous thrombosis should be performed for both hereditary and acquired thrombophilic defects. The family studies suggest that the presence of a lupus anticoagulant may be more clinically significant than elevated ACA in risk assessment. Although screening family members when the proband carries factor v Leiden is controversial, psychological reassurance of those who test negative and simple advice on occupations or social habits (e.g., smoking) for those who test positive may be important benefits.
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keywords = thrombosis
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12/336. Relapsing catastrophic antiphospholipid antibody syndrome: a mimic for thrombotic thrombocytopenic purpura?

    SUMMARY: The catastrophic antiphospholipid antibody syndrome (CAPS) is an uncommon disorder characterized by widespread micro- and macrovascular changes due to intravascular thrombosis. This complication of the antiphospholipid antibody syndrome is often fatal and recurrences are very rare. The differential diagnosis of CAPS includes thrombotic thrombocytopenic purpura (TTP) and this distinction may be difficult, but essential, for appropriate therapy. plasmapheresis is effective in both conditions, but anticoagulation, a mainstay in the treatment of CAPS, could be disastrous in TTP. We present the case of an elderly woman who survived two episodes of CAPS four years apart and whose clinical findings were also suggestive of TTP. The characteristics of TTP and CAPS are compared and the importance of accurate diagnosis is emphasized.
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keywords = thrombosis
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13/336. Intra-uterine death due to umbilical cord thrombosis secondary to antiphospholipid syndrome.

    This is a case of intra-uterine death due to a thrombotic event on the fetal side of circulation in a woman who was subsequently diagnosed with antiphospholipid syndrome.
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keywords = thrombosis
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14/336. aortic valve replacement for aortic regurgitation in a patient with primary antiphospholipid syndrome.

    A 42-year-old woman with the diagnosis of aortic regurgitation was admitted to hospital for surgical treatment. Ten years ago, primary antiphospholipid syndrome had been diagnosed, and she had a history of recurrent spontaneous abortions and deep vein thrombosis. She was suffering from moderate exertional dyspnea and chest pain. Catheter investigation revealed progressive dilatation of the left ventricle and a deterioration of the ejection fraction. The aortic valve was excised and replaced with a mechanical valve. A specimen of the aortic valve showed localized thickening and shrinkage of the midportion and base of each cusp, with vegetation on the surface. These localized, specific findings suggest that another mechanism may be involved in the cardiac valve pathology in patients with primary antiphospholipid syndrome. No hemostatic or thromboembolic problems were encountered after the surgery, and her postoperative course was uneventful.
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keywords = thrombosis
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15/336. Catastrophic arterial reactivity during primary antiphospholipid syndrome--a case report.

    Arterial reactivity leading to acute thrombosis at the site of a needle stick injury has never been described during antiphospholipid syndrome. The authors report a case characterized by a succession of thrombotic events occurring during or immediately after arterial angiographies or arterial surgery, in which catastrophic arterial reactivity can be strongly suspected. In this particular patient, it can be postulated that damage to the endothelial cells of the vessels injured during manipulation may have precipitated or aggravated the preexisting susceptibility to thrombosis.
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keywords = thrombosis
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16/336. Recurrent acute coronary events in a patient with primary antiphospholipid syndrome: successful management with intracoronary stenting.

    patients with antiphospholipid syndrome usually present with recurrent deep vein thrombosis, pulmonary thromboembolism and thromboembolic stroke. Recurrent coronary events, though reported, are rare. We describe an unusual case of antiphospholipid syndrome who presented with recurrent acute ischaemic events in two different coronary territories, who was managed successfully with intracoronary stenting.
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ranking = 0.21601513697187
keywords = thrombosis, coronary
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17/336. An IgG antiprothrombin antibody enhances prothrombin binding to damaged endothelial cells and shortens plasma coagulation times.

    OBJECTIVE: To test the hypothesis that some lupus anticoagulants are antiprothrombin antibodies, and that such antibodies enhance prothrombin binding to endothelial cells (EC) and thus promote clotting on the cell surface. methods: We generated a monoclonal antiprothrombin antibody (designated IS6) from a patient with primary antiphospholipid syndrome (APS). The antibody was analyzed for its binding properties, lupus anticoagulant activity, and pathophysiologic activity, using an EC-based plasma coagulation assay. RESULTS: IS6 is the first patient-derived monoclonal IgG antiprothrombin antibody. It bound to prothrombin with low affinity, reacted with 3 phospholipids (cardiolipin, phosphatidylethanolamine, and phosphatidylserine), and showed lupus anticoagulant activity. Moreover, IS6 enhanced the binding of prothrombin to damaged EC and shortened the EC-based plasma coagulation times. CONCLUSION: These findings suggest that IS6 may promote coagulation in areas of damaged EC in the host, and thus contribute to thrombosis in patients with APS.
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keywords = thrombosis
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18/336. A case of antiphospholipid antibody syndrome diagnosed after thrombosis of an arteriovenous shunt.

    A 32-year-old male dialysis patient with lupus nephritis was admitted because of shunt obstruction. The arteriovenous fistula was reconstructed, but obstruction recurred twice within several hours after surgery. A high blood level of anticardiolipin beta2-glycoprotein I antibody suggested that shunt obstruction was caused by a thrombotic tendency related to the antiphospholipid antibody syndrome. Accordingly, for the third shunt procedure, antiplatelet therapy (which had been commenced for systemic lupus erythematosus) was combined with dalteparin sodium from before surgery and warfarin was added postoperatively. This regimen prevented shunt obstruction. In conclusion, hemodialysis patients who suffer repeated shunt obstruction should be examined for antiphospholipid antibody syndrome.
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keywords = thrombosis
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19/336. Primary antiphospholipid syndrome associated with pernicious anaemia.

    AIM: To determine whether there is an association between pernicious anaemia and antiphospholipid antibody syndrome. methods: Fifteen patients with pernicious anaemia and 11 patients with iron deficiency anaemia (controls) were evaluated for clinical parameters of thrombosis and/or the presence of antiphospholipid antibodies, antinuclear antibodies or lupus anticoagulant. RESULTS: One asymptomatic patient with pernicious anaemia had laboratory features suggestive of the antiphospholipid syndrome. An additional three patients in each group had slightly elevated antiphospholipid antibody concentrations, within one standard deviation of the normal range. CONCLUSION: The association between pernicious anaemia and antiphospholipid antibody syndrome is rare and evaluation should be guided by clinical indications.
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keywords = thrombosis
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20/336. Hughes syndrome associated with cytomegalovirus infection.

    We report the case of a patient with an acute cytomegalovirus (CMV) infection who developed Hughes syndrome, manifested by a common iliac vein thrombosis. IgM anticardiolipin antibodies (aCL) appeared with the onset of the infection, followed later by IgG aCL. Five months later, both IgM and IgG aCL levels disappeared from the serum. This is the second case of Hughes syndrome associated with CMV infection to be reported in the literature.
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keywords = thrombosis
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