Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS).

Idiopathic myelofibrosis may be accompanied by portal hypertension. The authors report a 56-year-old man with idiopathic myelofibrosis and splenomegaly complicated by hepatopathy, severe portal hypertension and recurrent variceal bleeding. A transjugular intrahepatic porto-systemic shunt (TIPS) was inserted. Variceal bleeding never recurred. A short episode of encephalopathy, which is a known complication of porto-systemic shunting, ceased promptly after conservative treatment. The patient eventually died six months later due to metabolic deterioration and hepatic failure related to his underlying hematological disease. TIPS is a promising treatment modality for alleviating symptomatic portal hypertension in hematological disorders.