Portal hypertension secondary to myelofibrosis: a study of three cases.
BACKGROUND
In patients with idiopathic myelofibrosis (IM), portal hypertension (PHT) without thrombosis of the hepatic or splenoportal veins is infrequent.


OBJECTIVE
To ascertain the mechanisms responsible for the development of PHT in IM and their therapeutic implications.


METHODS
Color Doppler ultrasound with portal flow quantification, hepatic hemodynamic studies, and histological examinations of the liver biopsies were performed in three IM patients with PHT in whom hepatic and splenoportal thrombosis were ruled out.


RESULTS
Two patients showed sinusoidal PHT (increased hepatic venous pressure gradient), normal portal flow, and massive myeloid metaplasia of the liver. Transjugular intrahepatic portosystemic shunt (TIPS) was indicated for variceal bleeding and ascites unresponsive to medical therapy, and resulted in an adequate control of these PHT complications. At the time of TIPS placement, direct portal pressure measurement showed a marked presinusoidal component in the PHT. A third patient died as a consequence of the IM before treatment of PHT could be instituted. This patient showed an extremely increased portal flow and lesser hepatic infiltration.


CONCLUSIONS
IM patients with PHT might have a marked presinusoidal component contributing to PHT that is underestimated by hepatic vein catheterization. Treatment of the complications of PHT might not differ from that of patients with cirrhosis.
Alvarez-Larrán
Alberto
A
Hematology Department, Azienda Ospedaliero Universitaria di Careggi, Firenze, Italy.
Abraldes
Juan G
JG
Cervantes
Francisco
F
Hernández-Guerra
Manuel
M
Vizzutti
Francesco
F
Miquel
Rosa
R
Gilabert
Rosa
R
Giusti
Massimo
M
Garcia-Pagan
Juan Carlos
JC
Bosch
Jaime
J
D000368:Aged; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008102:Liver Circulation / Q000502:physiology; D008297:Male; D008875:Middle Aged; D011168:Portal System / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D055728:Primary Myelofibrosis / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D014463:Ultrasonography; D014655:Vascular Resistance / Q000502:physiology