SOS/TA-TMA

Abstract

 Both hepatic sinusoidal occlusion syndrome (SOS) and transplant-associated thrombotic microangiopathy (TA-TMA) are acquired thrombotic disorders in which sinusoidal endothelial cell damage and vascular endothelial cell damage are thought to be involved in their pathogenesis. In Japan, defibrotide (DF), a mixture of oligonucleotides made from porcine small intestinal mucosal DNA, has been available for use against SOS since 2019. although the mechanism of action of DF has not yet been fully elucidated, it is thought to exert its effects on SOS via endothelial protection, anticoagulation, and fibrinolysis-promoting effects. No effective treatment for TA-TMA has been established yet. However, recent studies have shown that complement activation may be involved in the pathogenesis of the disease, and it is attracting attention as a diagnostic marker and therapeutic target molecule.