Ricerc@Sapienza

Small hepatic veins Budd-Chiari syndrome is a rare disorder characterized by hepatic
venous outflow obstruction limited to the small intrahepatic veins, with normal appearance
of the large hepatic veins at imaging. In this case only a liver biopsy can demonstrate the
presence of a small vessels outflow block. Paroxysmal nocturnal haemoglobinuria (PNH)
is one of the most severe acquired thrombophilic state and represents one of the main
aetiological factors of Budd-Chiari syndrome. In patient affected by PNH with liver impairment
and/or ascites, Budd-Chiari syndrome must be always taken into consideration and,
if necessary, a liver biopsy performed to exclude the small hepatic veins involvement. We
report a case of small hepatic veins Budd–Chiari syndrome secondary to paroxysmal nocturnal
haemoglobinuria.