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APS - Hematology & Rheumatology Related


Thrombocytopenia in the Antiphospholipid Syndrome

SJS Aug 2003

Hypercoagulable State in Patients With Antiphospholipid Syndrome Is Related to High Induced Tissue Factor Expression on Monocytes and to Low Free Protein S

(Arteriosclerosis, Thrombosis, and Vascular Biology. 1996;16:1319-1326.) © 1996 American Heart Association, Inc.

Factor XIII in Primary Antiphospholipid Syndrome

PAUL R.J. AMES, LUIGI IANNACCONE, JOSE DELGADO ALVES, ANNAMARIA MARGARITA, LUIS R. LOPEZ, and VINCENZO BRANCACCIO J Rheumatol 2005;32:1058-62 Conclusion. Enhanced FXIII activity may contribute to atherothrombosis in primary APS via increased fibrin/fibrinogen cross-linking. This pathway is not exclusive to primary APS, being present also in thrombotic controls, but the presence of IgG aPL may favor a higher degree of FXIIIa activation in the primary APS group.

Testing for and Clinical Significance of Anticardiolipin Antibodies

Clinical and Diagnostic Laboratory Immunology, November 1999, p. 775-782, Vol. 6, No. 6 1071-412X/99/$04.00+0. The aCL assay is only one of the methods used to detect aPL, and the test should be administered with the LA and anti-B2GPI assays. The aCL assay is reasonably sensitive but not at all specific, with additional significant variation among laboratories and among commercial kits; therefore, clinicians should treat the clinical state and not an incidentally found antibody. Although there is an association between antibody titer and risk of thrombosis, this is not a ground for ignoring or not reporting weakly positive results. False-positive results that are difficult to interpret are particularly likely to occur when there are other causes of thrombosis such as atherosclerosis in the elderly; therefore, screening widely should not be encouraged. The predictive value of testing with the aCL ELISA for APS can be improved to an extent by concurrent LA testing, but often the appropriate duration and intensity of treatment after a first manifestation cannot be determined by these two tests. The anti-B2GPI ELISA offers an improved predictive value, but there is a need for an optimization or evaluation procedure to manipulate and assess the effects of the different variables on test performance. We suggest that laboratories interested in aPL evaluate the assay, both in an in-house form using an optimal buffer and a large quantity of B2GPI and in a commercial form when thoroughly tested and externally validated kits become available.

Anticardiolipin Antibodies (ACA), IgA, IgG, IgM


Ovarian Vein Thrombosis May Occur in Antiphospholipid Syndrome, May Be Underdiagnosed

Arthritis Rheum 2004 Jan;50:1:183-6. "Ovarian vein thrombosis in the antiphospholipid syndrome"

Primary antiphospholipid syndrome and panniculitis.

On-line Arch Rheumatol 1998; 2: 4 published on June 7, 1998

Antiphospholipid antibody testing: which are most useful for diagnosis?

Rheum Dis Clin North Am. 2006 Aug;32(3):455-63. Laboratory diagnosis of APS relies on the demonstration of a positive test for aPL. In clinical practice, the gold standard tests are those that detect beta2GPI-dependent aCL or LA. The question on the use of anti-beta2GPI asa routine diagnostic test remains unanswered, and testing for these antibodies should be only performed in very selected cases and not as an alternative to aCL or LA testing. Clinical utility and standardization are still lacking for other aPL specificities; therefore, their application as routine diagnostic tools is not recommended.

Thrombosis and the Antiphospholipid Syndrome

Hematology 2005 © 2005 The American Society of Hematology. Summary: Even with the most complete datasets, it is still important for the physician to develop a therapeutic plan appropriate for the individual patient, based on clinical presentation, co-morbid conditions, and other variables. With uncommon disorders and limited datasets, such as with the antiphospholipid syndrome, decision-making becomes even more difficult. Table 3 presents a strategy that the author uses when evaluating and developing a treatment plan for a patient with antiphospholipid syndrome and thrombosis, based on the available studies summarized in this article. Critical areas for future research include identifying which patients with antiphospholipid antibodies are at highest risk for thrombotic complications, developing new antithrombotic agents that are effective and safe, and investigating novel approaches to eliminate the autoantibody and, hopefully, the increased prothrombotic state.

Significance of Persistent Antiphospholipid Antibodies in the Elderly

Conclusion. Interpretation of a positive determination of APL is difficult in the elderly; persistent LAC may be the most valuable biological marker of APS in the elderly. (First Release July 1 2006; J Rheumatol 2006;33:1559–62)

Lupus anticoagulants

Update Date: 1/26/2005 Updated by: Rita Nanda, MD., Department of Hematology/Oncology, University of Chicago Medical Center, Chicago, IL. Review provided by VeriMed Healthcare Network.

Testing for and Clinical Significance of Anticardiolipin Antibodies

Clinical and Diagnostic Laboratory Immunology, November 1999, p. 775-782, Vol. 6, No. 6

Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients.

Ann Intern Med. 1992 Aug 15;117(4):303-8. CONCLUSIONS: Recurrent thrombosis is a potentially serious problem for patients with lupus anticoagulant or anticardiolipin antibodies or both. The site of the first event (arterial or venous) tended to predict the site of subsequent events. Intermediate- to high-intensity warfarin therapy may confer better antithrombotic protection than low- to intermediate-intensity warfarin therapy or aspirin therapy. Further studies are needed to define more precisely the rethrombosis rate and optimal type, intensity, and duration of antithrombotic therapy.

Anticardiolipin Antibodies (ACA): Antigenic Targets and Pathophysiology


New approaches to prevention of thrombosis in the antiphospholipid syndrome: hopes, trials, and tribulations.

Arthritis Rheum. 2003 Nov;48(11):3004-8.

Validation of the Sapporo criteria for antiphospholipid syndrome.

Arthritis Rheum. 2000 Feb;43(2):440-3. Some patients with false-negative results were true seronegative cases. CONCLUSION: The Sapporo criteria for APS compare favorably with the American College of Rheumatology criteria for SLE and are usable for clinical studies.

Patients with antiphospholipid antibodies and venous thrombosis should receive long term anticoagulant treatment

Annals of the Rheumatic Diseases, 1993, Vol 52, 689-692

LJP 1082: a toleragen for Hughes syndrome

Lupus, Vol. 13, No. 5, 335-338 (2004). DOI: 10.1191/0961203304lu1022oa. © 2004 SAGE Publications

Laboratory diagnosis and management challenges in the antiphospholipid syndrome.

Lupus. 2006;15(3):172-8. The antiphospholipid syndrome (APS) is characterized by recurrent arterial and/or venous thrombosis and pregnancy morbidity manifested by early or late losses. Laboratory diagnosis ofAPS relies on the demonstration of a positive test for antiphospholipid antibodies (aPL). In clinical practice, the gold standard tests are those that detect anticardiolipin antibodies (aCL) and/or the lupus anticoagulant (LA). Although other specificities for aPL have been described their clinical utility and standardization has still to be established. Persistence of aPL positive tests must be demonstrated, and other causes and underlying factors considered. Although it is universally recognized that the routine screening tests (aCL and/or LA) might miss some cases, careful differential diagnosis and repeat testing are mandatory before the diagnosis of 'seronegative APS' can be made. Correct identification of patients with APS is important, because prophylactic anticoagulant therapy can prevent thrombosis from recurring, and treatment of affected women during pregnancy can improve fetal and maternal outcome.

Anticardiolipin Test and the Antiphospholipid (Hughes) Syndrome: 20 Years and Counting!

© 2004. The Journal of Rheumatology Publishing Company Limited.

Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies

Annals of the Rheumatic Diseases 2004;63:730-736 © 2004 by BMJ Publishing Group Ltd & European League Against Rheumatism

RESULTS OF A RANDOMIZED, PLACEBO CONTROLLED, DOUBLE BLIND PHASE 1/2 CLINICAL TRIAL (RCT) TO ASSESS THE SAFETY AND TOLERABILITY OF LJP 1082 IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME

Arash Horizon, Michael H. Weisman, Daniel J. Wallace, Joan T. Merrill, Matthew D. Linnik, Keith A. Cockerill, Richard Furie. Conclusion: LJP 1082 appeared to be well tolerated in patients with antibodies to beta-2-GPI following a single intravenous dose up to 200 mg. Although the single-dose trial design was not intended to measure B cell tolerance, the observed binding of LJP 1082 to target antibodies was consistent with its intended pharmacological activity. The results of this trial support the further development of a B cell toleragen to treat Antiphospholipid Syndrome.

Antiphospholipid Antibody Syndrome: Lupus Anticoagulants and Anticardiolipin Antibodies


Patient information: Blood clotting problems due to antibodies (antiphospholipid antibody syndrome)

Peter H Schur, MD Harvard Medical School UpToDate performs a continuous review of over 330 journals and other resources. Updates are added as important new information is published. The literature review for version 13.2 is current through April 2005; this topic was last changed on July 6, 2004. The next version of UpToDate (13.3) will be released in October 2005.

The Presence of Multiple Prothrombotic Risk Factors Is Associated with a Higher Risk of Thrombosis in Individuals with Anticardiolipin Antibodies

J Rheumatol 2003;30:2385-91. Conclusion. In individuals with positive aCL-IgG, we observed an association between the number of prothrombotic risk factors and history of thrombotic events.


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