CATASTROPHIC-ANTIPHOSPHOLIPID-SYNDROME - RESPONSE TO REPEATED PLASMAPHERESIS OVER 3 YEARS

The catastrophic antiphospholipid syndrome (CAPS) is rare and usually fatal, In this report, we describe an unusual patient who, 31 years af ter experiencing an atypical preeclampsia-eclampsia presentation known today as the HELLP syndrome (hemolysis, elevated liver enzymes, and l ow platelets), developed CAPS, which seemed to complicate a diagnosis of primary antiphospholipid syndrome. She responded to repeated plasma pheresis over 3 years. Anticoagulants, corticosteroids, intravenous ga mma globulin, and intravenous cyclophosphamide had all failed to halt the progression of CAPS, but repeated plasmapheresis not only halted t he condition, but it led to the reversal of a leukoencephalopathy. The relationship between HELLP syndrome and CAPS is discussed, and possib le pathogenetic mechanisms that explain the efficacy of repeated plasm apheresis in this setting are suggested. It is postulated that perhaps plasmapheresis, through removal of cytokines or other mediators, disr upts the interaction between phospholipid-protein complexes and endoth elial cells. Repeated plasmapheresis should be considered in the most refractory cases of CAPS when more conventional treatment regimens hav e failed.