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Semin Thromb Hemost 2014; 40(08): 860-865
DOI: 10.1055/s-0034-1395158
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

The Paradox of the Lupus Anticoagulant: History and Perspectives

Vittorio Pengo
1   Department of Clinical Cardiology, University Hospital, Padova, Italy
,
Elisa Bison
1   Department of Clinical Cardiology, University Hospital, Padova, Italy
,
Gentian Denas
1   Department of Clinical Cardiology, University Hospital, Padova, Italy
,
Seena Padayattil Jose
1   Department of Clinical Cardiology, University Hospital, Padova, Italy
,
Alessia Bracco
1   Department of Clinical Cardiology, University Hospital, Padova, Italy
,
Alessandra Banzato
1   Department of Clinical Cardiology, University Hospital, Padova, Italy
› Author Affiliations
Further Information

Publication History

Publication Date:
11 November 2014 (online)

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Abstract

A unique coagulation inhibitor prolonging whole-blood clotting time was described more than 50 years ago in two patients with systemic lupus erythematosus (SLE). The immunoglobulin nature of the inhibitor and its interaction with antiphospholipid antibodies was later demonstrated and the term “lupus anticoagulant (LA)” was coined to describe this laboratory finding. It soon became apparent that LA was a misnomer as it is often found in plasma from patients with clinical conditions other than SLE and is associated with thromboembolic events that may occur in otherwise healthy individuals. Individuals with LA have circulating autoantibodies that inhibits blood coagulation. These are mostly of IgG or IgM class and mainly directed against a phospholipid (PL)-binding plasma protein, β2-glycoprotein I (β2GPI). The presence of β2GPI-dependent LA represents a well-recognized risk factor for venous and arterial thromboembolism, as well as pregnancy loss and morbidity. β2GPI-dependent LA in the presence of documented previous thromboembolism, or history of pregnancy loss/morbidity, identifies definite anti-PL syndrome. Laboratory diagnosis of LA is thus of particular importance, as it may assign patients with a common event (thrombosis) to a group with a high risk for recurrence, which is a prerequisite for long-term oral antithrombotic treatment.

Keywords

lupus anticoagulant - β2-glycoprotein I - phospholipids - syndrome - thrombosis - pregnancy
 

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