Usefulness of liver stiffness measurement in predicting hepatic veno-occlusive disease development in patients who undergo HSCT

Hepatic veno-occlusive disease (VOD), or sinusoidal obstructive syndrome (SOS), is a clinical syndrome characterized by hepato-megaly, ascites, weight gain and jaundice, which can develop more frequently in the first 30 days after hematopoietic stem cell transplantation (HSCT). Its incidence, although in fluenced by diagnostic criteria, has been estimated to be 13.7% (range 0 – 62.3%) and, in untreated hepatic severe VOD/SOS, it is associated with > 80% mortality. In this syndrome, sinusoidal endothelial cells and hepatocytes in zone 3 of the hepatic acinus are damaged by toxic metabolites generated during the conditioning regimen. The classic VOD pathway develops by the narrowing of the sinusoids, embolization of endothelial cells and increased clot formation, leading to obstruction of the sinusoids, subendothelial and centro-acinar fibrosis and then to portal- central fibrosis resulting in post-sinusoidal portal hypertension, which dominates the clinical picture.