Catastrophic APL Syndrome (CAPS) (Asherson's Syndrome) is a serious complication antiphospholipid syndrome (APLS). Many blood clots occur almost simultaneously and cause failure of multiple organs (multiorgan failure).
- Three or more organs involved (kidney: s-creatinine more than 50% increased (as evidence of kidney failure).
- Development (fast) over less than a week
- Histologically (tissue sample) carcass vasculitis with occlusion (blood clot) in at least one organ
- Lupus anticoagulant or anti-cardiolipin Antibodies
Safe CAPS: all 4 points, 3 points: probable CAPS (reference: Ascherson R, 2003)
- Intensive care unit
- Anticoagulation (Heparin and Marevan)
- Plasmapheresis (Plasma exchange)
- Cyclophosphamide (Sendoxane)
- Frozen plasma
- Off label treatment (for special cases): eculizumab
Serious condition. Deaths reported at 48%