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.
Bĕlohlávek
J
J
Department of Internal Medicine IV, 1st School of Medicine, Charles University, Prague, Czech Republic. jbelo@vfn.cz
Schwarz
J
J
Jirásek
A
A
Krajina
A
A
Polák
F
F
Hrubý
M
M
D001706:Biopsy; D004932:Esophageal and Gastric Varices / Q000209:etiology; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000209:etiology / Q000601:surgery*; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011170:Portasystemic Shunt, Surgical / Q000009:adverse effects / Q000379:methods*; D055728:Primary Myelofibrosis / Q000150:complications* / Q000601:surgery; D013163:Splenomegaly / Q000209:etiology; D016896:Treatment Outcome