2023 ACR/EULAR antiphospholipid syndrome classification criteria.

Objective: To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR.

Methods: This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and literature review; (2) Phase II, criteria reduction by modified Delphi and nominal group technique exercises; (3) Phase III, criteria definition, further reduction with the guidance of real-world patient scenarios, and weighting via consensus-based multicriteria decision analysis, and threshold identification; and (4) Phase IV, validation using independent adjudicators' consensus as the gold standard.

Results: The 2023 ACR/EULAR APS classification criteria include an entry criterion of at least one positive antiphospholipid antibody (aPL) test within 3 years of identification of an aPL-associated clinical criterion, followed by additive weighted criteria (score range 1-7 points each) clustered into six clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and two laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti-β-glycoprotein I antibodies).

Comparison of coagulation monitoring using ROTEM and Sonoclot devices in cardiac surgery: a single-center prospective observational study.

Background: Viscoelastic tests (VETs) are recommended during cardiac surgery to monitor coagulation status and guide transfusion. We compared the results of two VETs, the Sonoclot Analyzer and the ROTEM Sigma. Agreement between viscoelastic tests' subdiagnoses and overall diagnosis severity was assessed.

Inverted erythrocyte membranes demonstrate β2GPI-antiphospholipid antibody interactions and membrane crosslinking.

Introduction: The antiphospholipid syndrome (APS) is an acquired autoimmune disorder predisposing patients to thrombosis or pregnancy complications. Since inverted erythrocyte membranes (iEMs) might provide a physiologically relevant source of anionic phospholipids, we studied the interactions of phospholipid-binding proteins and APS antibodies using iEMs.

Materials & Methods: iEMs were prepared from packed erythrocytes by hypotonic lysis.

Dilute Russell's viper venom time reagents in lupus anticoagulant testing: a well-considered choice.

Background: Lupus anticoagulant (LAC) detection represents diagnostic challenges among which the multitude of available reagents and interference by anticoagulant treatment. One of the two advised tests is the dilute Russell's viper venom time (dRVVT). However, it is currently not clear whether all dRVVT reagents may be considered equivalent.

Differences in lupus anticoagulant final conclusion through clotting time or Rosner index for mixing test interpretation.

Background: Lupus anticoagulant (LAC) testing includes a screening, mixing and confirmation step. Although recently published guidelines on LAC testing are a useful step towards standardization, a lack of consensus remains whether to express mixing tests in clotting time (CT) or index of circulating anticoagulant (ICA). The influence of anticoagulant therapy, e.

Isolated acquired factor VII deficiency: review of the literature.

Objectives: Isolated acquired factor VII (FVII) deficiency is a rare haemorrhagic disorder. We report what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of acquired FVII deficiency.

Methods: We performed a literature search and included all articles published between 1980 and August 2015.

Fondaparinux as an alternative to vitamin K antagonists in haemodialysis patients.

Background: Accelerated vascular calcification and increased risk of calciphylaxis can be a reason to restrict the use of vitamin K antagonists in dialysis patients. We describe the use of fondaparinux, a prototype indirect factor Xa inhibitor, as an alternative anticoagulant to coumarin derivatives in dialysis patients.

Methods: In this case series, we included six chronic haemodialysis patients treated with vitamin K antagonists.

Antiphospholipid antibodies: evaluation of the thrombotic risk.

The laboratory diagnosis of the antiphospholipid syndrome (APS) via antiphospholipid antibody (aPL) tests, including lupus anticoagulant (LAC), anti-cardiolipin (aCL), or anti-beta2 glycoprotein I (aβ2GPI) antibodies remains a challenge. Coagulation tests for LAC as well as solid phase assays for aCL and aβ2GPI have methodological shortcomings, although for LAC large progress have been made in standardization. All assays are associated with clinical APS-criteria (thrombotic and/or pregnancy complications) but with limited specificity.

Evaluation of a new silica clotting time in the diagnosis of lupus anticoagulants.

Introduction: A new commercial silica clotting time (SCT), the HemosIL SCT assay (Instrumentation Laboratory, Milan, Italy) was evaluated in the laboratory diagnosis of lupus anticoagulants (LAC). This integrated test system for screening and confirmation was compared with the frequently used aPTT-based PTT-LA and Staclot-LA (Diagnostica Stago, Asnières, France) in a patient population investigated for LAC and in a subpopulation who met the clinical criteria for antiphospholipid syndrome (APS).

Materials And Methods: 201 samples were analysed with the HemosIL SCT assay.

Evaluation of new commercial enzyme-linked immunosorbent assay kits in the laboratory diagnosis of antiphospholipid syndrome in view of the revised classification criteria of the antiphospholipid syndrome.

Amendments to the Sapporo criteria for the diagnosis of the antiphospholipid syndrome (APS) have recently be published and include testing for the presence of IgG and IgM beta2-glycoprotein I (beta(2)GPI) antibodies. The Asserachrom Antiphospholipid antibodies line (Diagnostica Stago) with a monoclonal based standardisation, was evaluated in a Lupus anticoagulant (LAC) positive (n = 138) and a LAC negative (n = 134) populations. The ELISA line consists of the Asserachrom APA Screen, the Asserachrom APA IgG,M and the Asserachrom anti-beta(2)GPI IgG and IgM.

A functional coagulation test to identify anti-beta2-glycoprotein I dependent lupus anticoagulants.

Introduction: Lupus Anticoagulants (LAC) activity due to beta(2)-glycoprotein I (beta(2)GPI) antibodies shows a high correlation with thrombotic events. Since the binding of beta(2)GPI antibodies to phospholipids may be influenced by the final calcium chloride (CaCl(2)) concentration a discrimination between beta(2)GPI dependent LAC and beta(2)GPI independent LAC could be possible using clotting tests with various CaCl(2) concentrations and making them this way more sensitive to the presence of beta(2)GPI antibodies.

Materials And Methods: I evaluated the effect of 5 mM, 8.

Interpretation of normal plasma mixing studies in the laboratory diagnosis of lupus anticoagulants.

Introduction: Mixing studies are part of the laboratory diagnosis of lupus anticoagulants (LA). They are used to determine the evidence of an inhibitor by demonstrating persistence of an abnormal clotting time by mixing patient plasma with normal plasma. Since there is no standardised interpretation of results of mixing studies, percent correction formulas are proposed.

A 2-year retrospective analysis of laboratory testing for activated protein C resistance with a factor V-corrected activated partial thromboplastin time-based method.

The factor V-corrected activated protein C resistance assay is the test of choice to screen for the factor V Leiden mutation. During the past 2 years, local test results with the frequently used Coatest APCR kit were evaluated and compared with the results of DNA analysis, the 'gold standard'. Samples of 278 patients were analysed by both techniques.