Severe veno-occlusive disease after allogeneic bone marrow or peripheral stem cell transplantation--role of transjugular intrahepatic portosystemic shunt (TIPS).

Abstract

Veno-occlusive disease of the liver is a severe complication of allogeneic bone marrow or peripheral stem cell transplantation with a high mortality. In its severe form, the portal vein is used as an outflow tract for the arterial hepatic perfusion. A portosystemic side-to-side shunt, e.g. a transjugular intrahepatic portosystemic shunt, may facilitate portal outflow thus increasing hepatic (i.e. arterial) perfusion. The effect of a transjugular shunt on liver function and blood flow was studied in three patients receiving shunt treatment 0-2 days after the diagnosis of severe veno-occlusive disease occurring 28, 20, and 17 days after allogeneic transplantation for acute myeloid leukemia, Hodgkin's disease and chronic myeloid leukemia, respectively. The transjugular shunt reduced the portosystemic pressure gradient from 23 to 8, 18 to 5, and 33 to 13 mmHg in patients 1, 2, and 3, respectively, increased the stagnant portal vein flow to normal, and decreased the arterial resistive index, indicating an increase in the arterial perfusion of the liver. This was accompanied by rapid relief from abdominal pain and removal of ascites. The AST concentration dropped from 1230, 417, and 2930 U/l before to 93, 20, and 41 U/l and the PT-time ratio improved 3-7 days after shunt treatment while the bilirubin concentration continued to rise until the patients died 26, 42, and 33 days after transplantation from multiorgan failure (two patients) or intracerebral hemorrhage. The transjugular shunt may have improved abdominal and hepatic perfusion and prevented further necrosis of hepatocytes. It did not, however, affect jaundice or survival, which was limited by extrahepatic complications.