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)

Ancrod, which produces in vivo defibrination, has been shown to improve renal function and decrease fibrin deposition and crescents in experimental glomerulonephritis. Ancrod was given for 14 days to 5 patients with glomerulonephritis, moderate to severe renal functional impairment, crescents, and/or fibrin deposition in glomeruli. 4 patients had systemic lupus erythematosus. Ancrod treatment resulted in fibrinogen levels less than 50 mg/dl without bleeding, decrease of previously elevated factor VIII and von Willebrand factor levels, and normalization of in vitro platelet hyperaggregation. Renal function improved in all 5 patients. Serial renal biopsies showed a relatively rapid decrease of glomerular thrombi and necrosis and little increase in glomerular sclerosis. Ancrod administration appears safe, and may have a role in treatment of certain types of glomerulonephritis.
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PMID:Defibrination with ancrod in glomerulonephritis: effects on clinical and histologic findings and on blood coagulation. 681 68

The effects of an IgM lupus anticoagulant on coagulation tests and factor VIII related antigen (FVIIR:Ag) were investigated on a 62 year old man with lymphocytic lymphoma. He experienced no abnormal bleeding despite a prothrombin time of 29 seconds, PTT 84 seconds and inhibition of multiple coagulation factors. One course of chemotherapy induced a clinical response, normalization of coagulation studies and a rapid fall in IgM from 1120 to less than 30 mg%. Studies revealed a monoclonal IgM, immediate-acting lupus anticoagulant. This IgM could not be shown to affect normal FVIIIR:Ag but the patient's FVIIIR:Ag was grossly abnormal on crossed immunoelectrophoresis with a normal and a rapidly-migrating peak. The anodal component disappeared after chemotherapy. These studies suggest that the anticoagulant was temporally related to but did not cause the abnormality in FVIIIR:Ag. Such antibodies may occasionally serve as disease markers and may point out the disparity between coagulation tests and hemostasis.
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PMID:IgM anticoagulant with acquired abnormalities in factor VIII. 681 51

Five tests were carried out on 15 lupus anticoagulant plasmas: activated partial thromboplastin time with a commercial reagent (APTT); kaolin partial thromboplastin time with a human brain extract (KPTT-H); tissue thromboplastin inhibition test (TTI); Russell's viper venom time without phospholipid (RVVT); assay of phospholipid-related procoagulant activity (PPA). One of our criteria for diagnosis of lupus anticoagulant was a prolonged APTT; hence this test was abnormal in all 15 plasmas. An abnormal TTI was observed for the 15 lupus anticoagulants while PTT-H was abnormal in only 13 cases, RVVT in 11 cases and PPA assay in 12 cases. In another study evaluating the specificity of TTI and PPA assay, the TTI appeared to be influenced by factor II, V, VII, and X deficiencies, but not by factor VIII: C, IX, XI, and XII deficiencies, or by anti-factor VIII: C anticoagulants. Furthermore, the TTI displayed a weak sensitivity to heparin. On the other hand, the PPA assay was influenced by anti-factor VIII: C anticoagulants of high potency and was found to be more sensitive to heparin than the TTI. Our overall results emphasize the value of TTI as a screening test for the lupus anticoagulant.
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PMID:[Lupus anticoagulant screening: comparison of 5 tests (author's transl)]. 731 14

A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor VIII:c was 0.03 iu/ml with evidence of a slow-acting factor VIII inhibitor. Von Willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-DNA antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor VIII:c is less than 0.01 iu/ml and her factor VIII inhibitor titre is still > 20 Bethesda units/ml.
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PMID:Puerperal acquired factor VIII inhibitor causing a von Willebrand-like syndrome in a patient with anti-DNA antibodies. 767 76

Resistance to Activated Protein C (APC) was evaluated using 3 different methods: two of them were based on the prolongation of the Activated Partial Thromboplastin Time (APTT) using 2 different APTT reagents in the presence of APC, whereas the third method was based on the prolongation of prothrombin time when APC is added. The three methods were significantly correlated. APTT-based assays were sensitive to factor XII deficiency, whereas thromboplastin-based assay was sensitive to factor VII deficiency (< 0.5 UI/ml), which surestimates the response to APC. In contrast, an increase in factor VIII (F. VIII) level is associated with a decreased response to APC, when APTT-based assays are used, whereas thromboplastin-based assay is unmodified. During pregnancy, a decreased response to APC is observed, which is not only due to the increase in F. VIII, since thromboplastin-based assay is also modified. In Protein S (PS) immuno-depleted plasma, the low response to APC is corrected by addition of free PS: the thromboplastin-based assay was the most sensitive one to PS deficiency. However, in patients with congenital PS deficiency, there was no correlation between APC-resistance and free PS level. In patients with lupus anticoagulant, discrepancies were observed between the 3 methods, but with a high frequency of low response to APC. For the 3 assays, there was a good differentiation and correlation between normal and pathological results, the thromboplastin-based assay being perhaps the most discriminating. However, 3 unrelated thrombophilic patients showed normal results using thromboplastin-based assay, although they were APC-resistant using APTT-based assays. For 2 patients, this discrepancy can be explained by high levels of F. VIII. For the last patient, an abnormal F. VIII, resistant to APC can be suspected.
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PMID:Resistance to activated protein C: evaluation of three functional assays. 781 60

Lupus anticoagulants (LAs) are acquired antiphospholipid antibodies, and the occurrence of LA is associated with an increased risk of developing thrombosis. In a population of 46 patients with LA with or without LA-associated thrombophilia, it was analyzed whether the concentration of LA could be correlated to the individual thrombotic risk in patients with LA. No significant difference was found in the concentrations of LA measured by routinely used functional and immunologic assays in patients with LA with thrombophilia when compared with patients with LA without thrombophilia. Inhibition of thrombomodulin (TM) activity by LA has been postulated to be one of the major pathogenic mechanisms causing thrombophilia in LA. Therefore the inhibition of endothelial cell-dependent TM activity by LA was analyzed by using a protein C (PC) activation assay. Reduced rates of PC activation were found in only 2 out of the 46 cases, ruling out that inhibition of TM activity is a common phenomenon in patients with LA. However, anionic phospholipids are necessary to ascertain the anticoagulant activity of activated PC (APC). To prove the hypothesis that the anticoagulant activity of APC is inhibited by LA, the anticoagulant response of purified APC added to LA-containing plasma was measured through the amount of factor VIII inactivation. Thirteen out of 14 patients with recurrent thrombotic events and 10 out of 19 patients with one single episode of thrombosis showed an APC response outside the mean--2 SD range of normal human controls. In contrast, among 13 patients with LA without symptoms, only one showed an abnormal APC response. From these data it is concluded that LA inhibits the APC anticoagulant activity and that this type of acquired APC dysfunction may contribute to the pathogenesis of LA-associated thrombophilia. Moreover, the APC anticoagulant response assay may prove to be a useful marker to identify patients with LA with a high thrombotic risk.
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PMID:Acquired protein C dysfunction but not decreased activity of thrombomodulin is a possible marker of thrombophilia in patients with lupus anticoagulant. 782 42

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

Scientific and Standardization Committee (SSC) of International Thrombosis and Hemostasis has acted its activity since 1955 for establishing international concept on terminology, methodology in the fields of blood platelets, coagulation and fibrinolysis. Among the reports from 15 sub-committee in 1993 meeting, some clinically interesting topics are reported. In von Willebrand factor (vWF) Subcommittee, new classification of von Willebrand disease including new variant of VFW which shows defect on factor VIII binding capacity, is proposed. In Control of Anticoagulation Subcommittee, the necessity of coagulation monitoring during low molecular weight heparin (LMWH) administration was discussed. For prophylaxis use, monitoring is unnecessary except patients having renal failure or high-low body weight. For the treatment of venous thrombosis once or twice monitoring every 10 days would be necessary. In Lupus anticoagulant (LA)/phospholipid dependent antibodies Subcommittee, results of 3rd international survey for LA sent to 38 laboratories in 16 countries including Japan were reported. Most laboratory used APTT and dRVVT simultaneously as screening tests. Sensitivity and specificity of confirmatory test for LA are compared; the best one was Staclot LA and the second was DVVT.
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PMID:[An international attempt for standardization on terminology and methodology in hemostasis and thrombosis]. 802 94

The detection of lupus anticoagulant is important in laboratory evaluation of patients with thrombotic tendencies. The aim of this workshop was to assess the effectiveness of Australian laboratories in detecting these antibodies and assess the tests used. Fourteen laboratories took part in the exercise, held as a Workshop of the National Meeting of Australian Medical Laboratory Scientists in 1990. Seven unknown plasmas were distributed for testing prior to the meeting. While 100% correctly identified 3 strong inhibitors and a moderate strength inhibitor, the detection rate for specific individual tests varied from 38-100%. The detection rate for 2 weak inhibitors varied from 0-50%. Of the most commonly performed tests the least sensitive was the dilute Russell's viper venom time and the most sensitive was the tissue thromboplastin inhibition test, however, the degree of sensitivity seemed dependent on the source of thromboplastin. In some laboratories the Kaolin clotting time, with variations, was more sensitive. All participants correctly identified a factor VIII inhibitor as not of the lupus type. The false positive detection rate was 0%. All but one of the participating laboratories used 2 or more phospholipid dependent tests for the analysis of lupus anticoagulant (LA) which is in keeping with current international guidelines.
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PMID:Detection of lupus anticoagulant--an Australian perspective. 816 99

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


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