Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 26-year-old male who suffered from thrombocytopenia, factor V-deficiency and punctuate hemorrhages during the past. On admission the patient presented with acute left iliac artery occlusion. The walking distance was reduced to 200 m. Laboratory findings showed the existence of a lupus anticoagulant (LAC). As a therapeutical attempt a systemic lysis with high doses of streptokinase (9,250,000 I.E.) was carried out which was followed by a successful catheter lysis (250,000 I.E. streptokinase). During lysis the patient didn't show any unusual hemorrhagic events. Arterial thrombosis together with a lupus anticoagulant can be treated with high doses of streptokinase.
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PMID:[Streptokinase therapy in a patient with lupus anticoagulant and thrombosis of the A. iliaca externa]. 251 21

A retrospective multicentre study, undertaken under the aegis of the French National Society of Internal Medicine, involved 200 subjects with acquired circulating anticoagulants; 130 were female and 77 were male; mean age was 45 +/- 23 years (range: 10 months to 80 years). Mean duration of follow-up was 23 months. In 130 subjects the anticoagulants were detected as a result of a systematic screening examination. The main overt clinical manifestations were haemorrhages, venous or arterial thrombosis and spontaneous abortion. Typing of the anticoagulant, performed in 166 cases, showed the presence of an antiprothrombinase in 141; this enzyme is not responsible for severe bleeding unless it is associated with other disorders of coagulation; less frequent were an anti-factor VIIIc (n = 16) and an anti-factor V (n = 2) anticoagulants. An underlying pathology was found in 172 subjects, including systemic lupus erythematosus (n = 60), induced lupus (n = 11), discoid lupus (n = 3), infection (n = 23), blood disease (n = 19), cancer (n = 15) and vasculitis (n = 15); other factors were pregnancy (n = 5) and medicines (n = 6). The anticoagulant disappeared spontaneously in 10 cases and in 33 of the 115 subjects treated. In subjects with lupus and in children under twelve years of age, an antiprothrombinase was regularly identified at typing.
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PMID:[Circulating anticoagulants excluding hemophilia. Multicenter survey undertaken under the aegis of the French National Society of Internal Medicine apropos of 207 cases]. 336 61

Clinical and laboratory experience with circulating lupus anticoagulant in 3 patients undergoing coronary artery bypass procedures is reported. This circulatory anticoagulant inhibits activation of prothrombin by the prothrombin activator complex (factor Xa, factor V, and phospholipid). The presence of lupus anticoagulant was initially detected because of a prolonged activated partial thromboplastin time and a normal or mildly prolonged prothrombin time. The 3 patients underwent uncomplicated coronary artery bypass grafting and experienced no abnormal bleeding postoperatively. The lupus anticoagulant is a rare cause of bleeding after open-heart surgery. It appears to be a problem only when an additional coagulation defect is present.
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PMID:Coronary bypass surgery in patients with circulating lupus anticoagulant. 392 5

Lupus anticoagulants are spontaneously occurring antibodies with specificity for negatively charged phospholipids. The plasma of a patient with such a polyclonal antibody of IgM type demonstrated low levels of factor VIII coagulant activity (VIII:C) and factors IX, XI and XII when analyzed by biologic clotting assays, whereas in immunochemical assays, normal levels of VIII coagulant antigen and factor IX were obtained. After immunoadsorption of patient plasma with anti-IgM Sepharose, normal biologic activities were demonstrated in clotting assays for VIII:C, factors IX, XI, and XII. The addition of the patient's isolated IgM to normal plasma resulted in grossly abnormal results in these coagulation assays, and a pattern similar to that of the patient's plasma was obtained. The inhibitory effect of the patient's lupus anticoagulant on blood coagulation was demonstrated also in platelet-rich plasma. The results of the clotting assays indicated that the anticoagulant inhibited several of the reactions in the blood coagulation cascade. The availability of purified components made it possible to demonstrate an inhibiting effect on the activation of prothrombin by factor Xa in the presence of isolated platelets, as well as in a system where purified factor V and well defined phospholipid vesicles were substituted for the platelets.
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PMID:Inhibition of platelet prothrombinase activity by a lupus anticoagulant. 640 49

The plasmas of six patients with prolonged activated partial thromboplastin times were studied in detail. In five of the six, the Russell's viper venom and prothrombin times were likewise prolonged. Five of the patients had documented systemic lupus erythematosus; one lacked the necessary criteria for this diagnosis. On quantitation, factor XI was decreased in all six; factors X and XII were diminished in five of the six. When tested for inhibitory activity, plasma from each of the patients prolonged the celite eluate inhibition test for factor XII and/or XI inhibition. In the formation of the Xa-V-phospholipid-Ca2+ complex (prothrombinase), factors X and Xa were inhibited to a greater degree than factor V or the phospholipid. Finally, each plasma was isofocused, the inhibitory fractions were identified and the clotting factor specificity of each inhibitory peak was determined. Fractions inhibitory against factors XI and XII isofocused with the IgG in each patient's plasma. Based on the data presented from these six patients, the "lupus inhibitor" is in fact a heterogeneous collection of inhibitors directed against factors XII, XI and X rather than a homogeneous entity.
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PMID:The lupus inhibitor: a study of its heterogeneity. 733 Aug 26

In Sweden, clinicians at the Karolinska Hospital in Stockholm interviewed and took blood samples from 81 women aged 18-52 to examine the incidence of an insufficient response to activated protein C (APC), an increased level of antibodies to anionic phospholipids (Pla), and the presence of the mutation in the factor V gene in women who developed thrombosis while using oral contraceptives (OCs), in OC users who developed thrombosis during other risk situations (pregnancy or delivery, surgery, idiopathic), and in non-users who had a history of thrombosis. Women who had thrombosis during OC use had fewer pregnancies before developing thrombosis (p 0.05) and fewer recurrences after the thrombotic event than women in the other two groups. Non-users had the highest proportion of thrombotic recurrences (26%). Pulmonary embolism occurred more often as a result of the thrombotic event during OC use than during pregnancy, delivery, or surgery (p 0.01). APC resistance occurred in 27% of all women. The normalized ACP (nACP) ratio ranged between 0.41 and 1.48. Women who developed thrombosis during OC use had a significantly lower APC resistance than the other two groups (p 0.05). APC resistance increased as did the recurrence of thrombotic events (14-42%). The mean nACP ratio was highest among women who developed thrombosis during OC use and lowest in non-users (0.94 vs. 0.72). 40% of all women had mutation in the factor V gene. All but one woman was heterozygous. This mutation was present in relatively the same proportion in all three groups. The frequency of mutation was greater than that with laboratory-identified APC resistance in women with a history of thrombosis during OC use (38% vs. 14%; p 0.025). Coagulation and genetic analyses were highly correlated (p = 0.001). Pla were present in all three groups at essentially the same levels. Yet lupus anticoagulant activity was more common in OC users who developed thrombosis during other risk situations than the other two groups (p 0.05). 12% of all women had APC resistance and Pla. These findings show a flexible APC response.
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PMID:Thrombotic risk factors and oral contraception. 766 78

Some researchers claim that lupus anticoagulant-positive plasma may cause a false-positive reaction in the test for activated protein C (APC) resistance, a hereditary thrombophilic state characterized by abnormal factor V, which frequently causes venous thrombosis. We investigated whether anti-beta 2-glycoprotein I antibody (aGPI), which has recently come to be regarded as an anti-cardiolipin antibody (aCL) itself, might have an effect on the APC resistance test.
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PMID:Resistance to activated protein C activity of an anti-beta 2-glycoprotein I antibody in the presence of beta 2-glycoprotein I. 778 85

Staclot LA is a hexagonal (II) phase phospholipid clotting assay used to confirm the presence of lupus anticoagulants (LA). However, there have been complaints that the procedure contains several incubation steps requiring 15 min of operator time. The authors were able to shorten this procedure to a single 5 min incubation without affecting assay sensitivity. Both procedures were performed on 45 known lupus anticoagulant positive specimens, 25 normal donors, eleven plasmas from patients with known factor VIII or factor V inhibitors and ten other specimens submitted for lupus anticoagulant or anticardiolipin antibody testing but without complete testing to confirm the presence of LA prior to testing with Staclot LA. Excellent agreement was observed between the two procedures with concurrence in 87 of 91 specimens (95.6%). Each method detected 39 of 45 LA positive specimens giving a sensitivity of 86.7%. This modification shortens technologist time by two-thirds without compromising assay sensitivity, which will allow for automation on commonly used coagulation analysers.
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PMID:Evaluation of a modified procedure for Staclot LA for the confirmation of lupus anticoagulants. 784 17

Lupus anticoagulants (LA) are immunoglobulins (IgG, IgM, IgA or a mixture) which interfere with in vitro phospholipid (PL) dependent tests of coagulation (e.g. APTT, KCT, dilute Russell Viper Venom Time). LA are heterogeneous; consequently, the laboratory diagnosis is difficult and relies on multiple tests. We have developed a sensitive and relatively specific confirmatory test system based on fractions of two snake venoms. Textarin, a protein fraction of Pseudonaja textilis venom (Australian Eastern brown snake), activates prothrombin in the presence of PL, factor V and calcium ions. Ecarin, a protein fraction of Echis carinatus venom, will activate prothrombin in the absence of any cofactors. The activation of prothrombin by Textarin yields thrombin while Ecarin yields meizothrombin. In the presence of LA, the Textarin time is prolonged and the Ecarin time is unaffected. The test results are reported as a ratio of Textarin/Ecarin times (abnormal greater than 1.3). We have evaluated this test system in the following patient populations: LA positive, therapeutically heparinized, stable oral anticoagulated, liver disease, routine preoperative, anticardiolipin antibody positive LA negative, hemophilia A, various other hereditary factor deficiencies or dysfunctional proteins, and specific inhibitors of factor V and factor VIII. The LA positive patients represented a mixed population of autoimmune disease, drug-induced and post-infectious states. Our findings indicate the sensitivity of the Textarin/Ecarin system in the confirmation of LA. In order to use the test system most effectively, it is recommended to incorporate polybrene with Textarin when evaluating heparinized samples. Factor V deficiency and specific inhibitors of factor V yielded, in some instances, false positive results.
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PMID:The Textarin/Ecarin ratio: a confirmatory test for lupus anticoagulants. 816 13

A New method for the detection of lupus anticoagulant was developed. Plasma factor V activities using tissue thromboplastin (simplastin auto-SA) and partial thromboplastin (platelin excel LS-LS) were measured simultaneously. Furthermore, the ratio of the two activities was calculated (SA/LS ratio) as a marker of lupus anticoagulant. The normal range of SA/LS ratio was 0.79-1.39 (mean +/- 3SD). The high SA/LS ratios were detected in all of 15 patients with positive lupus anticoagulant, in 3 (5.8%) of 52 SLE patients with normal activated partial thromboplastin time, in 2 (28.7%) of 7 patients with hemophilia, in 1 (33.3%) of 3 patients with factor VIII inhibitor and in 4 (3.3%) of 122 patients with various diseases, respectively. All of 15 patients with liver dysfunction, 54 patients under warfarin treatment and 8 patients under heparin treatment had normal SA/LS ratio.
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PMID:[Newly developed method by the use of factor Y assay for the detection of the lupus anticoagulant]. 836 Oct 39


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