Q: 37 year old female with established diagnosis of antiphospholipid syndrome presented with Crisis (catastrophic stage) with clinical signs of generalized thrombosis. Patient has previously failed treatment with high dose steroid. Patient was doing well on warfarin but due to her diet change, INR on presentation is 2.0 instead of target of 2.5 to 3. What are other options at this stage?
Answer: Plasmapheresis or IVIG - or combination of both
Catastrophic Antiphospholipid Syndrome (CAPS) is a subset of patients who develop end-organ damage as a result of thrombic microangiopathy. Treatment includes anticoagulants, steroids, and possibly IVIG and therapeutic plasma exchange. Albumin is advocated by some experts for use in plasma exchange as traditional FFP contains clotting factors, cytokines, and complement activation products which may worsen the "thrombotic storm".
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