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An Acute Disseminated Coagulopathy-Vasculopathy Associated With the Antiphospholipid Syndrome

E. Nigel Harris, MPhil, MD, DM; Kathleen Bos, MD
Arch Intern Med. 1991;151(2):231-233. doi:10.1001/archinte.1991.00400020007001.
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The occurrence of venous and arterial thrombosis, recurrent pregnancy losses, and thrombocytopenia in patients with antiphospholipid (aPL) antibodies has been well documented.1,2 Antiphospholipid antibodies are autoantibodies that bind a variety of negatively charged phospholipids, the best known of which is cardiolipin.1,2 Some authors3-6 suggest that patients with these antibodies may represent a separate disease entity termed the antiphospholipid syndrome (APS). In addition to thrombosis, recurrent pregnancy losses, and thrombocytopenia, reported clinical features include livedo reticularis and possibly other skin lesions,6 heart valve abnormalities,7,8 and a variety of neurologic disorders.9 Laboratory features include a persistently elevated anti-cardiolipin test, usually IgG isotype,3,10 and/or an unequivocally positive lupus anticoagulant test.2,14 About half of the patients with the APS seen by rheumatologists have systemic lupus erythematosus (SLE), but in nonrheumatologic practices, the percentage of patients with SLE is much lower.

Clinical features that should prompt an


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