Correlation between Primary Myelofibrosis and the Association of Portal Thrombosis with Portal-Biliary Cavernoma: US, MDCT, and MRI Features

Objective Myelofibrosis is a rare chronic myelolymphoproliferative disease and is associated with increased risk of venous thromboembolism. The objective of this study is to retrospectively evaluate patients with primary myelofibrosis who underwent abdominal US, MDCT and MRI, in order to identify the development of portal thrombosis and its correlation with portal-biliary cavernoma. Methods We evaluated 125 patients with initial diagnosis of primary myelofibrosis and nonspecific abdominal pain who had undergone US with color Doppler. In 13 patients (8 men, 5 females; age: 45–85), US detected portal thrombosis with associated portal-biliary cavernoma. All patients subsequently underwent contrast-enhanced MDCT and MRI and 4 patients MR-cholangiography. The correlation between primary myelofibrosis and portal thrombosis and cavernoma respectively was calculated using χ2 test. Results About 10% of patients with primary myelofibrosis preliminary evaluated with US had partial (8 pts) or complete (5 pts) portal thrombosis associated with portal-biliary cavernoma with a χ2 = 0. In all patients, US detected a concentric thickening of main bile duct (MBD) wall (mean value: 7 mm); color Doppler always showed dilated venous vessels within the thickened wall of the biliary tract. Contrast-enhanced CT and MRI confirmed thickening of MBD walls with their progressive enhancement and allowed better assessment of the extent of the portal system thrombosis. MR-cholangiography showed a thin appearance of the MBD lumen with evidence of ab extrinsic compression. Conclusions The evidence of portal thrombosis and portal-biliary cavernoma in 10% of the patients with primary myelofibrosis indicates a close correlation between the two diseases. In the detection of portal thrombosis and portal-biliary cavernoma, US with color Doppler is the most reliable and economical diagnostic technique while contrast-enhanced MDCT and MRI allow better assessment of the extent of the portal vein thrombosis and of the complications of myelofibrosis.