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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.
Keywordsprimary myelofibrosis - portal system - thrombosis - portal-biliary cavernoma - diagnostic imaging - ultrasonography
Article published online:
12 October 2020
© 2020. Indian Society of Gastrointestinal and Abdominal Radiology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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- 1 Vibert E, Azoulay D, Castaing D, Bismuth H. [Portal cavenorma: diagnosis, aetiologies and consequences]. Ann Chir 2002; 127 (10) 745-750
- 2 De Gaetano AM, Lafortune M, Patriquin H, De Franco A, Aubin B, Paradis K. Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR Am J Roentgenol 1995; 165 (05) 1151-1155
- 3 Grgurević I, Buljevac M, Kujundzić M, Vukelić-Marković M, Kardum D, Brkljacić B. Common bile duct wall thickening due to intramural varices diagnosed by colour Doppler ultrasound. Ultraschall Med 2006; 27 (05) 483-486
- 4 Saint JH. The epicholedochal venous plexus and its importance as a means of identifying the common duct during operations on the extrahepatic biliary tract. Br J Surg 1961; 48: 489-498
- 5 Hunt AH. Compression of the common bile-duct by an enlarging collateral vein in a case of portal hypertension. Br J Surg 1965; 52: 636-637
- 6 Novellas S, Chevallier P, Peroux JL, Bruneton JN. Rare localization of a portal cavernoma in the wall of the common bile duct. Clin Imaging 2004; 28 (02) 132-134
- 7 Denys A, Hélénon O, Lafortune M. et al. Thickening of the wall of the bile duct due to intramural collaterals in three patients with portal vein thrombosis. AJR Am J Roentgenol 1998; 171 (02) 455-456
- 8 Gupta S, Singhal A, Goyal N, Vij V, Wadhawan M. Portal biliopathy treated with living-donor liver transplant: index case. Exp Clin Transplant 2011; 9 (02) 145-149
- 9 Agarwal AK, Sharma D, Singh S, Agarwal S, Girish SP. Portal biliopathy: a study of 39 surgically treated patients. HPB (Oxford) 2011; 13 (01) 33-39
- 10 Chow L, Jeffrey Jr RB. Intramural varices of the bile duct: an unusual pattern of cavernous transformation of the portal vein. AJR Am J Roentgenol 1999; 173 (05) 1255-1256
- 11 Besa C, Cruz JP, Huete A, Cruz F. Portal biliopathy: a multitechnique imaging approach. Abdom Imaging 2012; 37 (01) 83-90
- 12 DeLeve LD, Valla DC, Garcia-Tsao G. American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology 2009; 49 (05) 1729-1764
- 13 Diaz E, Nahon S, Charachon A. et al. [Portal vein thrombosis associated with a myeloproliferative disorder, prothrombin G20210A mutation, antiphospholipid syndrome, with repermeation during anticoagulant therapy]. Gastroenterol Clin Biol 2001; 25 (05) 549-551
- 14 Xavier SG, Gadelha T, Pimenta G. et al. JAK2V617F mutation in patients with splanchnic vein thrombosis. Dig Dis Sci 2010; 55 (06) 1770-1777
- 15 Denninger MH, Chaït Y, Casadevall N. et al. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 2000; 31 (03) 587-591
- 16 Saliba W, Mishchenko E, Cohen S, Rennert G, Preis M. Association between myelofibrosis and thromboembolism: A population-based retrospective cohort study. J Thromb Haemost 2020; 18 (04) 916-925
- 17 Gregory SA, Mesa RA, Hoffman R, Shammo JM. Clinical and laboratory features of myelofibrosis and limitations of current therapies. Clin Adv Hematol Oncol 2011; 9 (09) , Suppl 22) 1-16
- 18 DeLario MR, Sheehan AM, Ataya R. et al. Clinical, histopathologic, and genetic features of pediatric primary myelofibrosis–an entity different from adults. Am J Hematol 2012; 87 (05) 461-464
- 19 Barbui T, Carobbio A, Cervantes F. et al. Thrombosis in primary myelofibrosis: incidence and risk factors. Blood 2010; 115 (04) 778-782
- 20 Cervantes F, Alvarez-Larrán A, Arellano-Rodrigo E, Granell M, Domingo A, Montserrat E. Frequency and risk factors for thrombosis in idiopathic myelofibrosis: analysis in a series of 155 patients from a single institution. Leukemia 2006; 20 (01) 55-60
- 21 Ogawa S, Doishita K, Yamazaki Y, Nakanuma Y, Ohta G. Primary myelofibrosis associated with portal venous thrombotic occlusion and cavernous transformation around the biliary tree. J Clin Gastroenterol 1987; 9 (01) 115-116
- 22 Hamidah NH, Farisah NR, Azlinda AB. et al. A study of JAK2 (V617F) gene mutation in patients with chronic myeloproliferative disorders. Clin Ter 2012; 163 (02) 109-113
- 23 Thiele J, Kvasnicka HM, Zankovich R, Diehl V. Relevance of bone marrow features in the differential diagnosis between essential thrombocythemia and early stage idiopathic myelofibrosis. Haematologica 2000; 85 (11) 1126-1134
- 24 Vannucchi AM. Management of myelofibrosis. Hematology (Am Soc Hematol Educ Program) 2011; 2011: 222-230
- 25 Chandra R, Kapoor D, Tharakan A, Chaudhary A, Sarin SK. Portal biliopathy. J Gastroenterol Hepatol 2001; 16 (10) 1086-1092
- 26 Aguirre DA, Farhadi FA, Rattansingh A, Jhaveri KS. Portal biliopathy: imaging manifestations on multidetector computed tomography and magnetic resonance imaging. Clin Imaging 2012; 36 (02) 126-134