Triple-positive antiphospholipid syndrome does not guarantee positivity in each lupus anticoagulant assay

J Thromb Haemost. 2023 Dec;21(12):3539-3546. doi: 10.1016/j.jtha.2023.08.009. Epub 2023 Aug 18.

Abstract

Background: Triple positivity for all 3 criteria antiphospholipid antibodies confers high risk of symptom development in carriers, and recurrence in antiphospholipid syndrome (APS). Most triple-positivity studies report lupus anticoagulant (LA) testing as positive without distinguishing between positivity with dilute Russell's viper venom time (dRVVT) and activated partial thromboplastin time (APTT) and single-assay positivity or only perform dRVVT. Single LA assay repertoires remain in use in some centers, which risks missing some triple positives. Positivity with both assays may identify higher risk.

Objectives: The aim of this study is to investigate the frequency of single LA assay positivity in triple-positive patients.

Methods: Three hundred forty-two triple-positive profiles from nonanticoagulated patients (237 APS, 45 systemic lupus erythematosus without APS symptoms, and 60 nonclinical criteria) were identified from laboratory databases and assessed for LA positivity by dRVVT and/or APTT.

Results: Seventy-three of 237 (30.8%) APS samples were LA-positive with 1 assay, 40/237 (16.9%) by dRVVT only, and 33/237 (13.9%) with APTT only. Nineteen of 45 (42.2%) were LA-positive with 1 assay in the systemic lupus erythematosus cohort; 12/45 (26.7%) with dRVVT only and 7/45 (15.5%) with APTT only. Thirty-three of 60 (55.0%) were LA-positive with 1 assay in the nonclinical criteria cohort; 24/60 (40.0%) with dRVVT only and 9/60 (15.0%) with APTT only. The most common solid-phase assay profile was elevated immunoglobulin G aCL and aβ2GPI.

Conclusion: Up to 55.0% of triple-positive samples were positive in 1 LA assay, representing significant potential for misdiagnosis and inappropriate management via single LA assay repertoires.

Keywords: activated partial thromboplastin time; antiphospholipid syndrome; dilute Russell’s viper venom time; lupus anticoagulant; triple positive.

MeSH terms

  • Antibodies, Antiphospholipid
  • Antiphospholipid Syndrome* / diagnosis
  • Blood Coagulation Tests
  • Humans
  • Lupus Coagulation Inhibitor
  • Lupus Erythematosus, Systemic* / diagnosis
  • Partial Thromboplastin Time
  • Prothrombin Time

Substances

  • Lupus Coagulation Inhibitor
  • Antibodies, Antiphospholipid