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Catastrophic APS
See McMaster RARE-Bestpractices clinical practice guideline on
diagnosis and management of the catastrophic antiphospholipid syndrome. Legault K1, et a J Thromb Haemost. 2018 Jun 7. l.
Definition: acute, multiple vascular occlusions associated with high titre aPL
Rare. Precipitated by infection, surgery. High mortality despite Rx (50%)
Management: plasma exchange, anticoagulation (argatroban), intravenous gammaglobulin, cyclophosphamide
Future management of thrombotic disease in APS
•Improving imaging –MR direct thrombus imaging, CT/VQ Spect
•Intervention radiology- stenting to relieve post thrombotic syndrome
•Ritoximab - no
•Stem cell transplantation- no •DOACs – probably not...
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