Hughes Syndrome: A Patient’s Guide

The Antiphospholipid Syndrome II
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Antiphospholipid Syndrome (APS) Awareness

IgM and IgA antibodies are poorly specific. Pregnancy failure may be due to thrombosis in the placental bed, although alternative pathogenic mechanisms may apply, and may explain the tendency to very early losses prior to placentation. The methodology for LA and solid phase aPL assays e. Samples should not be repeatedly thawed and refrozen. Preliminary routine coagulation tests are helpful in eliminating undiagnosed coagulopathies and anticoagulant treatment. Demonstration of the presence of an inhibitor by mixing tests.

Antiphospholipid syndrome: state of the art on clinical practice guidelines

Demonstration of the phospholipid dependence of the inhibitor. Two test systems of different principles should be employed to ensure that weak LA is detected and to improve specificity, though patients are regarded as having a LA if one test is positive. Clinical evidence based on associations with thrombosis suggests that the DRVVT has good utility and should be one of these tests.

A mixing test may be used to detect an inhibitor and a confirmatory step e. Mixing tests are a criterion for LA and improve the specificity.

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As with any delicate machine, the human body can be profoundly affected by its supply of vital running materials. Thus, the tendency for the blood to clot excessively has the potential to cut off the oxygen supply to any organ of the body. As with any delicate machine, the human body can be profoundly affected by its supply of vital running materials. Thus, the tendency for the blood to clot.

However, they introduce a dilution factor and may make weak LA samples appear negative. Whenever possible, this should be confirmed by testing a fresh sample. These values may be available from the manufacturer, but local validation is advised. The method for calculating the degree of correction in the confirm step that has been recommended by the manufacturer should be used, provided that this takes into account the NPP clotting time.

QC plasmas should be prepared in the same way as test samples. Commercial QC plasmas should be matched with the reagents and validated, as differences in buffering between plasmas and reagents can lead to erroneous results while platelet contamination of plasma pools will influence the sensitivity. Laboratories should also participate in an external quality assurance programme. A confirmatory step e. LA testing is not recommended in patients receiving vitamin K antagonists VKA because exclusion of a LA is problematic whilst the international normalized ratio INR is in the therapeutic range.

Performing screening and confirmatory steps on equal volume mixtures of patient and normal plasma may be informative. If the screening step on the mixture is abnormal, this may be taken as grounds for considering that an inhibitor is present and the confirmatory step will demonstrate phospholipid dependence. Due to the dilution effect, negative testing in mixing studies does not exclude the presence of a LA.

Antiphospholipid syndrome - Symptoms and causes - Mayo Clinic

Low dose subcutaneous unfractionated heparin and low molecular weight heparin LMWH have less effect on the DRVVT and most commercial reagents contain a heparin neutralizang reagent sufficient to cover prophylactic doses. Platelet neutralization procedures should be avoided in samples containing heparin due to the potential for false positive LA results Exner, Assays should be performed at several dilutions as poor parallelism indicates interference by the inhibitor and unreliable results.

Features of the antiphospholipid syndrome (APS)

This book is not yet featured on Listopia. J Autoimmun ; 92 :1— The antiphospholipid syndrome as a neurological disease. J Thromb Haemost — If this item isn't available to be reserved nearby, add the item to your basket instead and select 'Deliver to my local shop' at the checkout, to be able to collect it from there at a later date. Although thrombotic damage has been advocated to explain many neurologic manifestations, direct immune-mediated processes may also be involved Repeated low-dose courses of rituximab in SLE-associated antiphospholipid syndrome: data from a tertiary dedicated centre.

In this situation, using higher dilutions of the test sample can sometimes restore parallelism, but the standard curve must also be extended. It should be recognized that some patients with factor inhibitors may also have a LA. IQC may be performed using suitable normal or APS patient samples local or commercial , or humanized monoclonal antibody preparations.

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  • Antiphospholipid Syndrome (APS).

There is nothing to suggest that measuring IgM antibodies in patients with thrombosis adds useful information. In patients with thrombosis, measuring IgM antibodies does not add useful information 2B. In patients with pregnancy morbidity, the role of IgM antibodies is unclear 2C. Testing for IgA antibodies is not recommended 1B. Incidental detection of aPL is common, e.

Hughes Syndrome

Even when persistent, incidental antibodies have been thought to be associated with a low rate of thrombosis, e. We recommend that primary thromboprophylaxis should not be used in those incidentally found to have aPL 2B. Warfarin therapy carries a substantial risk of bleeding. Although the risk is greatest in the first weeks, it persists for the duration of exposure. The cumulative incidence of stroke in patients with antiplatelet treatment only was statistically significantly higher than that in patients receiving the combination of antiplatelet and anticoagulation therapy.

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  • Antiphospholipid antibody syndrome (Hughes syndrome).

The authors suggested a larger study with more patients would be warranted. In the general stroke population, aspirin plus dipyridamole, or clopidogrel alone, are superior to aspirin alone.


Routine screening for aPL in patients with ischaemic stroke is not warranted 1B. For unselected stroke patients with a single positive aPL test result, antiplatelet therapy and warfarin are equally effective for preventing recurrent stroke 1B and antiplatelet therapy is preferred on grounds of convenience. It is a rare condition that may complicate established APS or present de novo. There are no data from randomized trials to inform treatment, which is based upon the thrombotic features and autoimmune background.

Immunomodulatory therapies including plasmaphaeresis, intravenous human IgG, corticosteroids and rituximab have been employed. As in all subjects with thrombosis, attention should be paid to modifiable risk factors such as smoking, obesity and exogenous female hormone use. Two subsequent prospective randomized trials have challenged this. Most manufacturers list APS as a specific exclusion to their use.

A baseline PT should be performed; if this is prolonged, an alternative PT reagent for which the baseline is normal should be used 1C. Antithrombotic interventions are used to reduce the incidence of pregnancy complications. Thus, while such therapy may be considered, based on an extrapolation from recurrent pregnancy loss evidence, at present this practice is not supported by the limited evidence available. In general, treatment should begin as soon as pregnancy is confirmed. While the advice and information in these guidelines is believed to be true and accurate at the time of going to press, neither the authors, the British Society for Haematology nor the publishers accept any legal responsibility for the content of these guidelines.

All authors contributed to the search for papers, interpretation of data, and drafting the paper and all approved the submitted final version.

Antiphospholipid syndrome (APS) - NHS

APS can lead to stroke, heart attack, kidney damage, deep vein thrombosis, pulmonary embolism, recurrent early miscarriages, premature birth due to eclampsia and late pregnancy losses. Antiphospholipid aPL antibodies can manifest as one of three types: lupus anticoagulant, anticardiolipin antibodies or antibeta2 glycoprotein I antibodies.

Identifying which type of aPL the patient has can help determine the risk of blood clots and the proper course of treatment. There is a great heterogeneity among studies on the laboratory and clinical criteria used to define APS and the treatment approaches used over the past four decades. We've noticed that you're using an ad blocker Our content is brought to you free of charge because of the support of our advertisers.

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