Intravenous Unfractionated Heparin vs. Therapeutic Plasma Exchange in Patients with Autoimmune Disease with Acute Thrombotic Events: Sampling in a Case of Catastrophic Antiphospholipid Syndrome

A variety of autoimmune disorders are associated with an increased risk of thrombosis. Previous studies have suggested combined therapy of heparin and therapeutic plasma exchange (TPE) with fresh frozen plasma (FFP) as the replacement fluid is beneficial in some cases of acute flare-up of autoimmune diseases complicated by thrombotic events. Nevertheless, it remains unknown whether clinicians do more harm than good by exposing patients to a "thrombotic storm" through simultaneous administration of heparin and the clotting factors in the FFP during TPE. A variety of data are currently available on therapeutic interventions for autoimmune diseases complicated with acute thrombosis; however, there is limited evidence on the exact efficacy of each individual approach and combinations of these measures. Herein, we report a case of catastrophic antiphospholipid syndrome (CAPS) to highlight the difficulty of therapeutic decision-making when complicated interactions occur between heparin and TPE. To our knowledge, this is the first case report of a patient diagnosed with CAPS successfully treated with a novel therapeutic strategy of escalating the heparin dosage when performing TPE by monitoring the partial prothrombin time to reduce the risk of the progression of thrombosis.