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Catastrophic Coagulation: A Fatal Clinical Vignett ...
Catastrophic Coagulation: A Fatal Clinical Vignette
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This case report describes a 30-year-old woman with triple-positive antiphospholipid syndrome (APS), a rare autoimmune disorder that causes arterial and venous thrombosis through antiphospholipid antibodies. Triple-positive APS refers to the presence of lupus anticoagulant, anti-β2 glycoprotein, and anticardiolipin antibodies and is associated with a particularly high risk of clotting. The most severe form, catastrophic antiphospholipid syndrome (CAPS), can cause rapid, widespread thrombosis and has a mortality rate of up to 50%.<br /><br />The patient presented after a gastrointestinal illness with vomiting, hematochezia, and progressive altered mental status. She had been taking apixaban, aspirin, and hydroxychloroquine at home. Evaluation revealed cardiogenic shock from right ventricular failure, nonbacterial thrombotic endocarditis on echocardiography, complete occlusion of the right popliteal artery, and later bilateral large middle cerebral artery infarcts on CT angiography. These findings raised strong concern for CAPS.<br /><br />Management included IV heparin, vasopressors, intubation, VA ECMO, pulse-dose corticosteroids, plasmapheresis, and plans for rituximab. She also underwent right popliteal balloon angioplasty, thrombectomy, and fasciotomy. Despite intervention, she had persistent thromboses, including deep venous clots in the right common femoral and saphenous veins, and ultimately died after the family chose withdrawal of care.<br /><br />The report highlights the complexity of anticoagulation in APS. Warfarin is generally first-line because it reduces thrombotic events better than direct oral anticoagulants (DOACs) such as apixaban. DOACs may be used in lower-risk patients or when warfarin is not tolerated, but this case underscores that even with aspirin and hydroxychloroquine, catastrophic thrombosis can still occur.
Asset Subtitle
Elle Maureen Newcome
Meta Tag
Author List
Elle Maureen Newcome, Kristen Young
Category
Clinical Vignettes
Concept
Antiphospholipid Antibody Syndrome
Concept
Catastrophic Antiphospholipid Antibody Syndrome
Concept
Antiphospholipid Antibody
Concept
Triple-Positive Status
Concept
Warfarin
Distinguished
Non-Finalist
Presenter Organization
University of Arizona College of Medicine Phoenix Internal Medicine Residency Program
Presenting Author
Elle Maureen Newcome
Track
Adult
Keywords
triple-positive antiphospholipid syndrome
catastrophic antiphospholipid syndrome
arterial thrombosis
venous thrombosis
nonbacterial thrombotic endocarditis
right ventricular failure
VA ECMO
plasmapheresis
apixaban
warfarin
Antiphospholipid Antibody Syndrome
Catastrophic Antiphospholipid Antibody Syndrome
Antiphospholipid Antibody
Triple-Positive Status
Warfarin
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