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)

Plasma from a patient with early manifestations of disseminated lupus erythematosus, a prolonged partial thromboplastin time with kaolin, mildly prolonged prothrombin time, and a circulating inhibitor affecting the assay of several clotting factors was investigated. The most sensitive test for the inhibitor was found to be the Russell viper venom time without phospholipid. A decrease in phospholipid concentration as well as decreased sodium chloride levels both significantly enhanced the effect of the inhibitor in several coagulation tests. Of various phospholipid substitutes tested phosphatidyl ethanolamine was the most effective in partially correcting for the inhibitor. The inhibitor was not localized to the patient's platelets, which were also found to partially neutralize its effect. Since lupus erythematosus is sometimes accompanied by thrombocytopenia the coagulation disorder may be aggravated by such a deficiency of phospholipid. The inhibitor appears to act by preventing binding of phospholipid to the Xa/V/thromboplastin complex. It was characterized as a gamma globulin of mixed class.
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PMID:Studies on phospholipids in the action of a lupus coagulation inhibitor. 122 21

Antiphospholipid antibodies (APA) are a family of immunoglobulins that react with anionic phospholipids, or anionic phospholipids-protein complexes. Recent evidence would support the latter definition. Lupus anticoagulants (LA) inhibit in vitro phospholipid dependent coagulation tests [e.g., activated partial thromboplastin time (APTT), prothrombin time (PT), and dilute Russell viper venom time (dRVVT)]. This inhibition appears to be specific for reagent phospholipids. The addition of freeze-thawed platelets or activated platelets will result in correction of the LA-induced abnormality. Anticardiolipin antibodies (ACA) are related to LA but appear to be distinct. ACA are detected by solid phase assays (ELISA, RIA) and require a plasma cofactor: beta 2 Glycoprotein-I (beta 2 GPI). ACA and LA activities can be separated in individual patient plasmas by affinity chromatography. In some instances they are of differing isotypes. Preliminary evaluation of beta 2 GPI in coagulation assays suggests it may function as a cofactor for LA activity. Recent work also suggests human prothrombin may represent a necessary cofactor for in vitro LA activity. Paradoxically, patients with LA/ACA may sustain thromboembolic events involving both venous and arterial sites. The prothrombotic properties of LA/ACA have not been satisfactorily characterized. A number of proposals have been reported, including inhibition of prostacyclin (PGI2) generation by endothelial cells, decreased activity of the protein C system, impaired fibrinolysis, and inhibition of beta 2GPI. Among these various hypotheses, down regulation of the protein C system appears most plausible. Also, LA/ACA may interfere with the phospholipase A2-phospholipid substrate complex involved in the generation of arachidonic acid from membrane phospholipids.
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PMID:Antiphospholipid antibodies: proposed mechanisms of action. 128 81

Lupus anticoagulant (LAC) is an acquired inhibitor of prothrombin activator complex, which probably interferes with the phospholipid portion. Characteristically, LAC prolongs the partial prothrombin time, but only slightly prolongs the prothrombin time. It is a paradoxical fact that LAC is characterized by thrombosis. It was initially described in patients with SLE, but recently, it has been described as occurring with other autoimmune disorders. Patients with LAC have been treated with steroid and aspirin, anti platelet agents or warfarin. Steroid and aspirin therapy has been reported useful for habitual abortion associated with LAC. In our study, 11 patients, whose prior pregnancies resulted in habitual abortion (41 abortions), received intentional prednisolone (40 mg/day) and aspirin (81 mg/day) therapy before further pregnancies. The doses of both agents were decreased gradually, and the therapy with prednisolone (10-15 mg/day) and aspirin (40.5 mg/day) was maintained during the pregnancy period. The outcome of pregnancy was successful in 7 out of 10 pregnancies. To evaluate the relationship between LAC and glomerulopathy, we examined the renal biopsy from 5 LAC cases without SLE. In pathological findings, there were 3 of with mild proliferative GN and 2 cases of minor glomerular abnormality. There were no characteristic findings in LAC nephropathy.
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PMID:[Lupus anticoagulant]. 130 2

The Lupus Anticoagulant (LA) is an immunoglobulin directed towards the phospholipid portion of the prothrombin activator complex. This immunoglobulin because first identified in the plasma of patients with Systemic Lupus Erythematodes, was named as Lupus Anticoagulant. Although initially described in patients with SLE, it was subsequently observed in other diseases and also in patients without any manifest disease. SLE or similar diseases are present in 35% of LA (+) patients. The LA prevalence in SLE patients has been found as 34% whereas ACA prevalence was found as 44%. While searching for the presence of LA and the levels of APA in cases having unexplained recurrent fetal losses, a family (a mother and 3 daughters) was discovered, whose each member has been diagnosed as SLE at different times and with different symptoms. Data suggesting the presence of LA and high APA levels were determined in all the members of the family and also it was realised that cause who had recurrent fetal losses had the highest APA levels. In addition to immunoassay methods to detect APA, examination of coagulation tests in patients with unexplained thrombosis and/or fetal losses, would be of great help.
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PMID:[Lupus anticoagulant and antiphospholipid antibody values in a family with systemic lupus erythematosus]. 141 67

To evaluate the usefulness of preoperative screening for coagulation disorders in children, we prospectively studied laboratory and bleeding histories in 1603 children undergoing tonsillectomy. All patients had preoperative laboratory screening with a complete blood count, prothrombin time, activated partial thromboplastin time, and bleeding time. Persistent abnormalities on repeat testing 1 week later were investigated further by a standardized schema. A subset of 129 patients, including all those who bled perioperatively or had laboratory abnormalities, completed a standard historical questionnaire. Thirteen patients had persistent laboratory abnormalities diagnostic of lupus inhibitor (5), non-lupus inhibitor (6), mild hemophilia A (1), and vonWillebrand disease (1). Two patients had persistently prolonged activated partial thromboplastin times of undefined cause. Fourteen patients (10.8%) interviewed reported positive bleeding histories. Of these, five, including the patient with vonWillebrand disease, had persistent laboratory abnormalities. History alone failed to detect the patient with hemophilia A. For patients with inhibitors or prolonged activated partial thromboplastin times of unknown cause, surgery was delayed until the coagulation abnormalities resolved, and there was no perioperative bleeding. The patient with vonWillebrand disease had severe postoperative bleeding despite treatment with cryoprecipitate. In predicting perioperative bleeding, history and laboratory screening had a high specificity but a very low positive predictive value due to poor sensitivity and a low prevalence of bleeding. Some children with bleeding disorders may be identified first during routine preoperative coagulation testing, and replacement therapy or delay or cancellation of surgery may reduce or prevent perioperative hemorrhage. However, the large number of false positive laboratory tests and bleeding histories, coupled with the relative rarity of inherited and acquired coagulopathies, raises doubts about the overall value of routine screening.
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PMID:Preoperative history and coagulation screening in children undergoing tonsillectomy. 851 74

Lupus anticoagulant was detected in 205 newly diagnosed, untreated patients with systemic lupus erythematosus by the following tests: kaolin clotting time, activated partial thromboplastin time, plasma prothrombin time, and, in the last 99 patients, by dilute Russell's viper venom time. In 10 patients, lupus anticoagulant was detected by kaolin clotting time prolongation, corrected by inosithin but not by normal plasma; 12 and 6 of them had prolonged activated partial thromboplastin time and partial plasma prothrombin time, respectively. Only 10 patients had a history of recurrent abortions and/or thrombosis, nine of whom had lupus anticoagulant as shown by the kaolin clotting time test. Of the 99 patients studied by all four tests, 9 showed lupus anticoagulant by both kaolin clotting time and dilute Russell's viper venom time; 7 had a history of abortion and/or thrombosis. The dilute Russell's viper venom time test is easy to perform and not affected by inhibitors to factor VIII or IX. It is recommended as a primary screening test for lupus anticoagulant detection in a hospital clinical laboratory.
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PMID:Evaluation of four coagulation tests to detect plasma lupus anticoagulants. 174 92

Primary lymphoma of the spleen is characterized by predominant splenomegaly. Lymphoplasmacytic malignant lymphoma of the spleen, of low malignancy in the Kiel classification, low and intermediate grade in the National Cancer Institute Working Formulation (NCIWF), is rare. It is often associated with a monoclonal immunoglobulin M (IgM). Four patients presenting with primary splenic lymphoma of plasmacytic type associated with a high level of monoclonal IgM and a lupus anticoagulant (LA) are described. This association has not previously been reported. In contrast with the usual heterogeneity of LA, this LA is relatively homogeneous with an important prolongation of the prothrombin time (greater than 18 sec for a control of 12), more prolonged partial thromboplastin time (PTT) of the mixture patient + control plasma than PTT of the patient plasma. Despite the important coagulation abnormalities, none of these four patients has presented any hemorrhagic or thrombotic complications, even during major surgery such as splenectomy. The lupus-like anticoagulant effect ran parallel with the monoclonal IgM. Survival, after splenectomy and chemotherapy, appears to be favourable: three patients are alive with survivals of greater than or equal to 7 years. The follow-up is as yet too short for the last patient.
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PMID:Lupus anticoagulant associated with primary malignant lymphoplasmacytic lymphoma of the spleen: a report of four patients. 174 35

Plasmas from 16 patients that were found to be positive both for anticardiolipin antibodies (ACA) and lupus anticoagulants (LA) were incubated with liposomes that contained anionic phospholipids. In 11 of these plasmas, ACA could be cosedimented with the liposomes in a dose-dependent manner, whereas LA activity of the remaining supernatant was unaffected. LA activity of purified total IgG from 6 patients was measured in three different coagulation tests, using normal plasmas from different species. Prolongation of the aPTT, KCT and dRVV clotting times was observed only with normal plasma from human origin, not with bovine, rat or sheep plasma. Highly purified coagulation factors Xa, Va and prothrombin, both of human and bovine origin, were used to establish for two patient IgG's the effect of LA on the rate of thrombin formation in the presence and absence of lipid vesicles composed of 20 mole% phosphatidylserine and 80 mole% phosphatidylcholine. A strong and dose dependent inhibition by LA was observed only when human prothrombin was used as substrate in the prothrombinase complex in the presence of lipids. No inhibition was found when bovine prothrombin was used as substrate. The inhibitory effect observed in the presence of human prothrombin was independent of the source of factors Xa and Va, and was not found in the absence of lipid. Preliminary binding studies suggest that LA only associate with a lipid surface, provided that human prothrombin and calcium ions are present. These data indicate that LA are not directed to phospholipids alone, but presumably recognize an epitope which becomes exposed upon Ca(2+)-mediated binding of human prothrombin to phospholipids.
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PMID:Lupus anticoagulant IgG's (LA) are not directed to phospholipids only, but to a complex of lipid-bound human prothrombin. 179 7

The relation between lupus anticoagulant (LA) and repeated abortions was evaluated in a case-controlled study of 50 women with 3 or more unexplained spontaneous abortions compared with 50 control subjects who had 2 or more normal pregnancies and no previous spontaneous abortion or fetal deaths. LA was detected in 5 of 50 cases (10%, 95% confidence limits 1.69% to 18.31%) but in none of the 50 controls. There was an indication that missed abortions may be more frequent in LA positive women. The women who had recurrent abortions but were LA negative had prolonged prothrombin time values (though within normal range) compared to the control group. LA being a treatable cause of idiopathic recurrent abortion should be sought for in women with unexplained fetal losses.
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PMID:Study of lupus anticoagulant in pregnant women with recurrent abortion. 179 44

In 45 patients with diabetes mellitus (DM) without cerebro-cardiovascular diseases (CCVD) the modified method of the tissue thromboplastin inhibition test (TTIT) was studied. TTIT is the method of detection of the lupus anticoagulant (LA), LA, first recognized in patients with systemic lupus erythematosus, is presented by a prolonged activated partial thromboplastin time (APTT), a slightly to moderately prolonged prothrombin time (PT), and high incidence of biological false-positive seroreactions for syphilis (BFP). In patients with LA, thrombotic events have been reported. Six of the 45 diabetic patients were TTIT-positive (13.3%). All control subjects were TTIT-negative. In the TTIT-positive diabetics APTT and PT were normal. BFP also were not observed. The difference between LA and these results in TTIT-positive diabetics remains unclear. Clinical profiles except for duration of DM between the TTIT-negative and TTIT-positive diabetics did not differ. Follow-up studies may resolve an association between the results of TTIT and DM.
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PMID:The tissue thromboplastin inhibition test in diabetics without cerebro-cardiovascular diseases. 180 39


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