Cases reported "Antiphospholipid Syndrome"

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1/80. Recurrent thrombo-embolic episodes: the association of cholangiocarcinoma with antiphospholipid syndrome.

    antiphospholipid syndrome is a disorder of recurrent vascular thrombosis, pregnancy loss and thrombocytopenia associated with persistently elevated levels of antiphospholipid antibodies. It was first described in a group of patients with systemic lupus erythematosus but has since been associated with a wide range of conditions, including other autoimmune disorders and malignancy. It can also occur in isolation, the so-called primary antiphospholipid syndrome. We describe an elderly woman with the antiphospholipid syndrome thought to be associated with a cholangiocarcinoma.
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keywords = pregnancy
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2/80. Two cases of autoimmune thrombocytopenic purpura associated with antiphospholipid antibodies.

    We report 2 cases of autoimmune thrombocytopenic purpura associated with antiphospholipid antibodies. In the first case the titer of antiphospholipid antibodies was neither related to disease activity nor influenced by immunosuppressive therapy. In the second, cerebral infarction occurred in spite of severe thrombocytopenia. Our results provide additional evidence that antiphospholipid antibodies are not involved in the pathogenesis of autoimmune thrombocytopenic purpura. Nevertheless, the frequency and clinical significance of this association would suggest that patients with autoimmune thrombocytopenic purpura should be tested for antiphospholipid antibodies, particularly before pregnancy or surgical procedures.
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keywords = pregnancy
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3/80. Immunological abnormalities in primary APS evolving into SLE: 6 years follow-up in women with repeated pregnancy loss.

    We have performed a prospective study to determine the prevalence of immunological abnormalities and the evolution from primary antiphospholipid syndrome (APS) into systemic lupus erythematosus (SLE) in women who had had unexplained repeated pregnancy loss (PL) and APS. Of 105 women with abortions or fetal deaths, 33(31%) fulfilled criteria for APS. Among these patients with primary APS, 24% had antinuclear antibodies (ANA), 91% had elevated circulating immune complexes (CIC), 70% had low total haemolytic complement (CH100), 52% had low levels of complement 4 (C4) and 30% had low levels of complement 3 (C3), in a significantly higher prevalence than women whose pregnancies were successful (control group). Through out a 6 y follow-up, 3 (9%) of the patients with APS who had autoimmune related abnormalities when entered into the study developed features of lupus like disease (LLD) or fullblown SLE. Our findings suggest that women with unexplained repeated PL with APS who presented with positive ANA, high levels of CIC, low levels of CH100, C3 and C4, may define a subset of patients exhibiting immunological alterations similar to those of SLE. These parameters may help in the assessment of prognosis in APS patients with PL. Those patients should be carefully surveyed with regard to the development of connective tissue diseases.
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ranking = 5
keywords = pregnancy
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4/80. Laparoscopic splenectomy in pregnancy.

    A laparoscopic splenectomy during pregnancy is described in this case report. The operation took place at 18 weeks' gestation for life-threatening thrombocytopaenia secondary to antiphospholipid syndrome that had failed to respond to medical therapy. The patient made a full and rapid recovery and was delivered of a healthy baby girl at term.
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ranking = 5.0882782680855
keywords = pregnancy, gestation
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5/80. Widespread cutaneous necrosis occurring in association with the antiphospholipid syndrome: a report of two cases.

    The antiphospholipid syndrome is an acquired prothrombotic state where thrombosis and/or pregnancy loss is related to the presence of antiphospholipid antibodies. Cutaneous necrosis secondary to intravascular thrombosis of small dermal vessels is a recognized but rare association with antiphospholipid syndrome. We report two patients with high circulating levels of anticardiolipin antibodies who developed widespread cutaneous necrosis as the first clinical manifestation of the antiphospholipid syndrome. The exact mechanism by which antiphospholipid antibodies mediate thrombosis is uncertain; however, proposed mechanisms of activity include endothelial cell activation, altered endothelial production of prostacyclin, activation of platelets, and modulation of the protein c and S pathways.
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keywords = pregnancy
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6/80. ovulation induction and early pregnancy loss in a woman susceptible to autoimmune diseases: a possible interrelationship.

    We report a 34-year-old woman with sequentially occurring autoimmune diseases that are possibly triggered by numerous ovulation inductions. At the ages of 26-32 years, she experienced 27 uncontrolled ovulation induction cycles using clomiphene citrate (CC) or CC plus human menopausal gonadotropin plus human chorionic gonadotropin. She became pregnant at the ages of 27, 30 and 31 with subsequent pregnancy loss in the 28th, 8th and 10th week of gestation, respectively. insulin-dependent diabetes mellitus (IDDM) developed at the age of 28. During the second year of ovulation induction, at the age of 27, she developed arthralgia that worsened and became migratory from the age of 31. thrombocytopenia appeared at the age of 33. The diagnosis of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) was established at the age of 34. To the best of our knowledge, this is the first case of concurrent IDDM, SLE and APS in a patient associated with ovulation inductions. Excessive levels of estradiol achieved during the ovulation inductions could play a role in the expression of multiple autoimmune diseases in the susceptible woman.
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ranking = 5.0882782680855
keywords = pregnancy, gestation
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7/80. Mitral valve plasty in systemic lupus erythematosus in the setting of antiphospholipid syndrome.

    The first case was of a 27-year-old female, who was diagnosed as having mitral valve stenosis with regurgitation, systemic lupus erythematosus and antiphospholipid syndrome at her previous pregnancy. We performed mitral valve plasty, which included open mitral commissurotomy and Kay's annulo plasty. The second case was of a 53-year-old female, who was diagnosed as old myocardial infarction, mitral regurgitation, systemic lupus erythematosus and antiphospholipid syndrome. She underwent mitral valve plasty and coronary artery bypass grafting. Both cases were treated by administration of methylpredonisolone and heparin perioperatively to avoid thrombosis and aggravation of systemic lupus erythematosus. Both patients showed good postoperative outcome without complications. We consider that it is important to perform the plasty as far as possible, and to administer effective anticoagulation treatment to prevent complications for patients in the setting of systemic lupus erythematosus and antiphospholipid syndrome.
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ranking = 1
keywords = pregnancy
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8/80. Vena cava thrombosis associated with nephrotic syndrome in the puerperal gestational cycle.

    CONTEXT: The puerperal gestational cycle is accompanied by a state of physiological hypercoagulability. Thromboembolic phenomena may occur at this time. OBJECTIVE: To report on a clinic case involving a patient that presented a family history of thromboembolism and developed deep vein thrombosis in a lower limb and vena cava thrombosis during the puerperal gestational cycle, displaying nephrotic syndrome as the main complication. DESIGN: Case report.
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ranking = 0.52966960851306
keywords = gestation
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9/80. Successful delivery in a pregnant woman with lupus anticoagulant positive systemic lupus erythematosus treated with double filtration plasmapheresis.

    The case was a 29 year old female who has suffered from systemic lupus erythematosus (SLE) since 15 years of age. The activity of SLE was low, and she took prednisolone orally. Her first pregnancy failed after 14 weeks. In the second pregnancy, she had thrombocytopenia, prolonged activated partial thromboplastin time (APTT), positive lupus anticoagulant (LAC) and thus was diagnosed with antiphospholipid antibody syndrome (APS). Combination therapy with steroids and aspirin was started, and she underwent treatment of double filtration plasmapheresis (DFPP) in the early stage of pregnancy. Her platelet count increased, and the value of APTT has normalized with DFPP treatment. She delivered successfully on the 32nd week of pregnancy. We think that DFPP is an effective and safe treatment in patients with an LAC positive pregnancy.
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ranking = 5
keywords = pregnancy
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10/80. bone marrow necrosis and refractory hellp syndrome in a patient with catastrophic antiphospholipid antibody syndrome.

    We describe a 22-year-old woman who developed extensive and fatal bone marrow necrosis along with involvement of liver, lung, and central nervous system during pregnancy in the background of very high titers of antiphospholipid and anti-beta2 glycoprotein antibodies. This case illustrates a rarely recognized, potentially fatal complication of aPL in the setting of pregnancy.
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ranking = 2
keywords = pregnancy
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