Portal Vein Thrombosis and Budd-Chiari Syndrome as the Inital Symptom of Polycythemia Vera and Hyperhomocysteinemia
,
Abstract
Portal Vein Thrombosis (PVT), commonly associated with cirrhosis of liver and thrombophilia, is one of the causes of severe abdominal pain. In the absence of non-cirrhotic non-malignant extrahepatic portal vein thrombosis, Myeloproliferative Disease (MPD) and an underlying thrombotic disorder should always be suspected and investigated. Hyperhomocysteinemia has been well-documented to increase the risk of arterial thrombotic events, peripheral arterial disease, and stroke. It is also a risk factor for deep-vein thrombosis. In the general population, association with portal vein thrombosis is very unusual, and only a few cases have been reported. We describe a case of Polycythemia Vera (PV) and hyperhomocysteinemia presenting with severe abdominal pain due to portal vein thrombosis. The patient underwent phlebotomy and was prescribed life-long anticoagulant, aspirin, vitamin B6, vitamin B12, and folic acid, then referred to a hematologist.
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[14] Berk PD, Goldberg JD, Silverstein MN, Weinfeld A, Donovan PB, Ellis JT, et al. Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil therapy. New England Journal of Medicine. 1981; 304(8):441-7. [DOI:10.1056/ NEJM198102193040801] [PMID]
[15] “Leukemia and Hematosarcoma” Cooperatve Group, European Organizaton for Research on Treatment of Cancer (EORTC). Treatment of polycythaemia Vera by radiophosphorus or busulphan: A randomized trial. Britsh Journal of Cancer. 1981; 44(1):75-80. [DOI:10.1038/bjc.1981.150] [PMID]
[16] Landolf R, Marchioli R, Kut J, Gisslinger H, Tognoni G, Patrono C. et al. Efcacy and safety of low-dose aspirin in polycythemia vera. New England Journal of Medicine. 2004; 350(2):114-24. [DOI:10.1056/NEJMoa035572] [PMID]
[17] Najean Y, Rain JD. Treatment of polycythemia vera: The use of hydroxyurea and pipobroman in 292 patents under the age of 65 years. Blood. 1997; 90(9):3370-7. [PMID]
[18] Najean Y, Rain JD. Treatment of polycythemia vera: Use of 32P alone or in combinaton with maintenance therapy using hydroxyurea in 461 patents greater than 65 years of age. The French Polycythemia Study Group. Blood. 1997; 89(7):2319-27. [PMID]
[19] Kiladjian JJ, Cassinat B, Chevret S, Turlure P, Cambier N, Roussel M, et al. Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera. Blood. 2008; 112(8):3065-72. [DOI:10.1182/ blood-2008-03-143537] [PMID]
[20] Kang SS, Wong PW, Malinow MR. Hyperhomocyst (e) inemia as a risk factor for occlusive vascular disease. Annual Review of Nutriton. 1992; 12(1):279-98. [DOI:10.1146/annurev.nu.12.070192.001431] [PMID]
[21] Bazzano LA, Reynolds K, Holder KN, He J. Effect of folic acid supplementaton on risk of cardiovascular diseases: A meta-analysis of randomized controlled trials. JAMA. 2006; 296(22):2720-6. [DOI:10.1001/jama.296.22.2720] [PMID]
[22] Wang X, Qin X, Demirtas H, Li J, Mao G, Huo Y, et al. Efcacy of folic acid supplementaton in stroke preventon: A meta-analysis. Lancet. 2007; 369(9576):1876-82. [DOI:10.1016/S0140-6736(07)60854-X]
[23] Mart-Carvajal AJ, Solà I, Lathyris D, Salant G. Homocysteinelowering interventons for preventng cardiovascular events. Cochrane Database of Systematc Reviews. 2014; (4):CD006612. [DOI:10.1002/14651858.CD006612.pub2] [PMID]
[2] Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, Hamid S, Hashizume M, et al. Consensus on extra-hepatc portal vein obstructon. Liver Internatonal. 2006; 26(5):512-9. [DOI:10.1111/j.1478- 3231.2006.01269.x] [PMID]
[3] Ögren M, Bergqvist D, Björck M, Acosta S, Eriksson H, Sternby NH. Portal vein thrombosis: Prevalence, patent characteristcs and lifetme risk: A populaton study based on 23 796 consecutve autopsies. World Journal of Gastroenterology (WJG). 2006; 12(13):2115- 9. [DOI:10.3748/wjg.v12.i13.2115] [PMID] [PMCID]
[4] Den Heijer M, Koster T, Blom HJ, Bos GM, Briët E, Reitsma PH, et al. Hyperhomocysteinemia as a risk factor for deep-vein throm bosis. New England Journal of Medicine. 1996; 334(12):759-62. [DOI:10.1056/NEJM199603213341203] [PMID]
[5] Ray JG. Meta-analysis of hyperhomocysteinemia as a risk factor for venous thromboembolic disease. Archives of Internal Medicine. 1998; 158(19):21029-34. [DOI:10.1001/archinte.158.19.2101] [PMID]
[6] Den Heijer M, Rosendaal FR, Blom HJ, Gerrits WB, Bos GM. Hyperhomocysteinemia and venous thrombosis: A metaanalysis. Thrombosis and Haemostasis. 1998; 80(12):874-7. [DOI:10.1055/s-0037-1615380] [PMID]
[7] Passamont F, Malabarba L, Orlandi E, Baratè C, Canevari A, Brusamolino E, et al. Polycythemia Vera in young patents: A study on the long-term risk of thrombosis, myelofbrosis and leukemia. Haematologica. 2003; 88(1):13-8. [PMID]
[8] Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F, et al. Survival and prognosis among 1545 patents with contemporary polycythemia vera: An internatonal study. Leukemia. 2013; 27(9):1874-81. [DOI:10.1038/leu.2013.163] [PMID] [PMCID]
[9] Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA, et al. Proposals and ratonale for revision of the World Health Organizaton diagnostc criteria for polycythemia vera, essental thrombocythemia, and primary myelofbrosis: Recommendatons from an ad hoc internatonal expert panel. Blood. 2007; 110(4):1092-7. [DOI:10.1182/blood-2007-04-083501] [PMID]
[10] Tefferi A. JAK2 mutatons in polycythemia vera--molecular mechanisms and clinical applicatons. New England Journal of Medicine. 2007; 356(5):444-5. [DOI:10.1056/NEJMp068293] [PMID]
[11] Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, et al. Philadelphia-negatve classical myeloproliferatve neoplasms: Critcal concepts and management recommendatons from European LeukemiaNet. Journal of Clinical Oncology. 2011; 29(6):761-70. [DOI:10.1200/JCO.2010.31.8436] [PMID] [PMCID]
[12] Marchioli R, Finazzi G, Specchia G, Cacciola R, Cavazzina R, Cilloni D, et al. Cardiovascular events and intensity of treatment in polycythemia vera. New England Journal of Medicine. 2013; 368(1):22- 33. [DOI:10.1056/NEJMoa1208500] [PMID]
[13] Tartaglia AP, Goldberg JD, Berk PD, Wasserman LR. Adverse effects of antaggregatng platelet therapy in the treatment of polycythemia vera. Seminars in Hematology. 1986; 23(3):172-6. [PMID]
[14] Berk PD, Goldberg JD, Silverstein MN, Weinfeld A, Donovan PB, Ellis JT, et al. Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil therapy. New England Journal of Medicine. 1981; 304(8):441-7. [DOI:10.1056/ NEJM198102193040801] [PMID]
[15] “Leukemia and Hematosarcoma” Cooperatve Group, European Organizaton for Research on Treatment of Cancer (EORTC). Treatment of polycythaemia Vera by radiophosphorus or busulphan: A randomized trial. Britsh Journal of Cancer. 1981; 44(1):75-80. [DOI:10.1038/bjc.1981.150] [PMID]
[16] Landolf R, Marchioli R, Kut J, Gisslinger H, Tognoni G, Patrono C. et al. Efcacy and safety of low-dose aspirin in polycythemia vera. New England Journal of Medicine. 2004; 350(2):114-24. [DOI:10.1056/NEJMoa035572] [PMID]
[17] Najean Y, Rain JD. Treatment of polycythemia vera: The use of hydroxyurea and pipobroman in 292 patents under the age of 65 years. Blood. 1997; 90(9):3370-7. [PMID]
[18] Najean Y, Rain JD. Treatment of polycythemia vera: Use of 32P alone or in combinaton with maintenance therapy using hydroxyurea in 461 patents greater than 65 years of age. The French Polycythemia Study Group. Blood. 1997; 89(7):2319-27. [PMID]
[19] Kiladjian JJ, Cassinat B, Chevret S, Turlure P, Cambier N, Roussel M, et al. Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera. Blood. 2008; 112(8):3065-72. [DOI:10.1182/ blood-2008-03-143537] [PMID]
[20] Kang SS, Wong PW, Malinow MR. Hyperhomocyst (e) inemia as a risk factor for occlusive vascular disease. Annual Review of Nutriton. 1992; 12(1):279-98. [DOI:10.1146/annurev.nu.12.070192.001431] [PMID]
[21] Bazzano LA, Reynolds K, Holder KN, He J. Effect of folic acid supplementaton on risk of cardiovascular diseases: A meta-analysis of randomized controlled trials. JAMA. 2006; 296(22):2720-6. [DOI:10.1001/jama.296.22.2720] [PMID]
[22] Wang X, Qin X, Demirtas H, Li J, Mao G, Huo Y, et al. Efcacy of folic acid supplementaton in stroke preventon: A meta-analysis. Lancet. 2007; 369(9576):1876-82. [DOI:10.1016/S0140-6736(07)60854-X]
[23] Mart-Carvajal AJ, Solà I, Lathyris D, Salant G. Homocysteinelowering interventons for preventng cardiovascular events. Cochrane Database of Systematc Reviews. 2014; (4):CD006612. [DOI:10.1002/14651858.CD006612.pub2] [PMID]
| Files | ||
| Issue | Vol 4 No 2 (2019): Spring | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/crcp.v4i2.1704 | |
| Keywords | ||
| Portal vein thrombosis Polycythemia vera Budd-Chiari syndrome Hyperhomocysteinemia | ||
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How to Cite
1.
Pazooki B, Radkhah H, Sherafat A. Portal Vein Thrombosis and Budd-Chiari Syndrome as the Inital Symptom of Polycythemia Vera and Hyperhomocysteinemia. CRCP. 2019;4(2):40-43.
