Recovery from catastrophic antiphospholipid syndrome by a plasma exchange procedure: report of four cases and review of the literature

Catastrophic Antiphospholipid Syndrome (CAPS) is a rare (1%), life-threatening variant of Antiphospholipid Syndrome (APS). It has been found that the recovery rate is best when the treatment protocol includes anticoagulants, steroids and therapeutic plasma exchange (TPE). The treatment of CAPS with TPE is not, however, well defined as procedure modalities have not yet been standardized, and the best replacement fluid for TPE is still a controversial issue. Although the most commonly used one, fresh frozen plasma (FFP), contains natural anticoagulants, it is also made up of clotting factors, complement activation products and cytokines which could worsen CAPSʹ “thrombotic storm”. The successful management of 4 CAPS patients, including TPE sessions initiated in the 1st week from diagnosis and using albumin solution as the replacement fluid, is described here. TPE was performed daily for the first 3 days, then tapered off, and withdrawn on the basis of patientʹs clinical condition. One of the patients was also treated with anticoagulants, while the others received anticoagulants plus high doses of steroids in addition to TPE. Our results indicate that, when initiated promptly and albumin solution is used as the replacement fluid, TPE can be considered an effective, safe treatment for CAPS.