肝臓の静脈疾患：何を指すのか？

Vascular diseases of the liver include damage to arterial and venous vessels, both hepatic and perihepatic. The main venous forms are Budd-Chiari syndrome (BCS), portal vein thrombosis (PVT) and porto-sinusoidal vascular disease (PSVD), defined by the location of the thrombotic obstruction. BCS corresponds to an obstruction of the hepatic veins, PVT to an obstruction of the portal vein with a risk of cavernoma, and PSVD to damage the intrahepatic microcirculation without cirrhosis. These rare diseases mainly affect young adults and cause portal hypertension in the absence of underlying liver disease. Myeloproliferative neoplasms are a common risk factor, among other systemic causes (coagulation disorders, antiphospholipid antibodies, infections, pregnancy) or local causes (inflammation, surgery).