Treatment of Sinusoidal obstruction syndrome (Veno-occlusive disease)

Abstract

 Sinusoidal obstruction syndrome (SOS), also called veno-occlusive disease (VOD) of the liver, is one of the most relevant complications of hepatic sinusoidal endothelial origin that appears early after haematopoietic cell transplantation (HCT). Despite its relatively low incidence and the fact that most cases of SOS/VOD resolve spontaneously, in the severe SOS/VOD that evolve to multi-organ failure have a mortality rate>80% and represent one of the major clinical problems after HCT. In these patients, the sinusoidal endothelial cells and hepatocytes are damaged by toxic metabolites generated by the conditioning regimen. Several risk factors have been identified for the development of SOS/VOD. Although defibrotide is recommended for both prevention and treatment, there is no satisfactory therapy for managing severe SOS/VOD. This review describes the new definition for late onset SOS/VOD diagnosis and the severity grading of suspected SOS/VOD from the European Society for Blood and Marrow Transplantation, the results of current treatment including the Japanese therapeutic use program, defibrotide treatment protocol.