A 'primitive' catastrophic antiphospholipid syndrome

A female patient affected by a thrombotic syndrome due to non SLE-related antiphospholipid antibodies (APA), developed a serious, non inflammatory, thrombotic macroangiopathy and uremic-hemolytic syndrome during oral anticoagulant therapy. She was treated with aspirin, dipyridamole i.v. and with a total of 14 fresh frozen plasma exchange (PEX), but the thrombotic syndrome only showed a partial response with the APA titre dropping considerably. Renal failure did not improve because of acute cortical necrosis. The patient died from infective complications.