Normalization of plasma factor X levels in amyloidosis after plasma exchange

Some patients with systemic light chain amyloidosis develop bleeding complications that can be caused by vascular infiltration with amyloid or by alterations of the coagulation or fibrinolytic systems. Factor X deficiency is the most common cause of bleeding manifestations, although deficiencies of other clotting factors, a disruption in the conversion of fibrinogen to fibrin, and circulating heparin-like anticoagulants have also been reported. Deficiency of factor X is a well-recognized cause of bleeding manifestations in patients with light chain amyloidosis. This acquired disorder appears to be secondary to adsorption of factor X to the amyloid fibrils. Previous studies have shown that infusion of plasma into patients with acquired factor X deficiency and amyloidosis induces a transitory improvement of the coagulation tests. However, there is a rapid return to pretransfusion levels. In this manuscript we report the clinical application of plasma exchange in the management of a patient with systemic light chain amyloidosis with acquired factor X deficiency.