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 CAPS' "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 TPE was performed daily for the first 3 days, then tapered off, and withdrawn on the basis of patient's clinical condition.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.