Using Preoperative Therapeutic Plasma Exchange in Lupus Anticoagulant Hypoprothrombinemia Syndrome to Achieve...

Lupus anticoagulant hypoprothrombinemia syndrome (LA-HPS) is a rare cause of acquired factor II deficiency associated with lupus anticoagulant due to antiprothrombin antibody, which predisposes to bleeding rather than thrombosis. We report a case of a 48-year-old female with LA-HPS resulting in a bleeding diathesis due to acquired prothrombin or factor II (FII) deficiency in the context of very high titers of anti-phosphatidylserine/prothrombin antibodies (anti-PS/PT). In preparation for elective mitral valve replacement surgery due to infective endocarditis, she received four sessions of therapeutic plasma exchange (TPE) preoperatively, followed by IVIG (2 g/kg total in divided doses). Her FII activity improved from 7% to 53% on the morning of her surgery, and her IgM anti-PS/PT decreased from greater than 150 units to a nadir of 26.4 units. She completed the surgery without bleeding complications. She has since been maintained on immunosuppression with mycophenolate and without bleeding. We illustrate one of the few reported successful uses of TPE to effectively remove the anti-PS/PT antibodies and optimizing hemostasis by increasing FII activity before a surgical procedure to mitigate the high bleeding risk. We also add to the limited existing evidence that immunosuppression is a reasonable long-term treatment in patients with anti-prothrombin antibodies causing severe hypoprothrombinemia with bleeding.